BioPAX pathway converted from "Peroxisomal protein import" in the Reactome database. Peroxisomal protein import 过氧化物酶体小细胞的细胞器bounded by a single membrane and contain variable compositions of proteins depending on cell type. Peroxisomes function in oxidation of fatty acids, detoxification of glyoxylate, and synthesis of plasmalogens, glycerophospholipids containing an alcohol with a vinyl-ether bond (reviewed in Lohdi and Semenkovich 2014). All of the approximately 46 proteins contained in peroxisomal matrix are imported from the cytosol by a unique mechanism that does not require the imported proteins to be unfolded as they cross the membrane (Walton et al. 1995, reviewed in Ma et al. 2011, Fujiki et al. 2014, Baker et al. 2016, Dias et al 2016, Emmanoulidis et al. 2016, Erdmann 2016, Francisco et al. 2017). The incompletely characterized process appears to involve the transport of the proteins through a variably sized pore in the membrane comprising at least PEX5 and PEX14 (inferred from the yeast homologs in Meinecke et al. 2010, the yeast pore is reviewed in Meinecke et al. 2016). Oligomeric proteins are also observed to cross the peroxisomal membrane (Otera and Fujiki 2012) but their transport appears to be less efficient than monomeric proteins (Freitas et al. 2011, inferred from mouse homologs in Freitas et al. 2015, reviewed in Dias et al. 2016).
In the cytosol, receptor proteins, PEX5 and PEX7, bind to specific sequence motifs in cargo proteins (Dodt et al. 1995, Wiemer et al. 1995, Braverman et al. 1997). The long and short isoforms of PEX5 (PEX5L and PEX5S) bind peroxisome targeting sequence 1 (PTS1, originally identified in firefly luciferase by Gould et al. 1989) found on most peroxisomal matrix proteins; PEX7 binds PTS2 (originally identified in rat 3-ketoacyl-CoA thiolase by Swinkels et al. 1991) found on 3 imported proteins thus far in humans. The long isoform of PEX5, PEX5L, then binds the PEX7:cargo protein complex (Braverman et al. 1998, Otera et al. 2000). PEX5S,L bound to a cargo protein or PEX5L bound to PEX7:cargo protein then interacts with a complex comprising PEX13, PEX14, PEX2, PEX10, and PEX12 at the peroxisomal membrane (Gould et al. 1996, Fransen et al. 1998, inferred from rat homologs in Reguenga et al. 2001).
The ensuing step in which the cargo protein is translocated across the membrane is not completely understood. During translocation, PEX5 and PEX7 become inserted into the membrane (Wiemer et al. 1995, Dodt et al. 1995, Oliveira et al. 2003) and expose a portion of their polypeptide chains to the organellar matrix (Rodrigues et al. 2015). One current model envisages PEX5 as a plunger that inserts into a transmembrane barrel formed by PEX14, PEX13, PEX2, PEX10, and PEX12 (the Docking-Translocation Module) (Francisco et al. 2017).
After delivering cargo to the matrix, PEX5 and PEX7 are recycled back to the cytosol by a process requiring mono-ubiquitination of PEX5 and ATP hydrolysis (Imanaka et al. 1987, Thoms and Erdmann 2006, Carvalho et al. 2007). PEX7 is not ubiquitinated but its recycling requires PEX5 mono-ubiquitination. A subcomplex of the Docking-Translocation Module comprising the RING-finger proteins PEX2, PEX10, and PEX12 conjugates a single ubiquitin to a cysteine residue of PEX5 (Carvalho et al. 2007, reviewed in Platta et al. 2016). The mono-ubiquitinated PEX5 and associated PEX7 are then extracted by the exportomer complex consisting of PEX1, PEX6, PEX26, and ZFAND6 (inferred from rat homologs in Miyata et al. 2012). PEX1 and PEX6 are members of the ATPases Associated with diverse cellular Activities (AAA) family, a group of proteins that use the energy of ATP hydrolysis to remodel molecular complexes. PEX1 and PEX6 form a hetero-hexameric ring, best described as a trimer of PEX1/PEX6 dimers (inferred from yeast in Platta et al. 2005, yeast homologs reviewed in Schwerter et al. 2017). Data on the yeast PEX1:PEX6 complex suggest that these ATPases use a substrate-threading mechanism to disrupt protein-protein interactions (Gardner et al. 2018). PEX7 is also then returned to the cytosol (Rodrigues et al. 2014). Once in the cytosol, ubiquitinated PEX5 is enzymatically deubiquitinated by USP9X and may also be non-enzymatically deubiquitinated by nucleophilic attack of the thioester bond between ubiquitin and the cysteine residue of PEX5 by small metabolites such as glutathione (Grou et al. 2012).
Defects in peroxisomal import cause human diseases: Zellweger syndrome, neonatal adrenoleukodystrophy, infantile Refsum disease and rhizomelic chondrodysplasia punctata types 1 and 5 (Barøy et al. 2015, reviewed in Nagotu et al. 2012, Braverman et al. 2013, Wanders 2014, Fujiki 2016, Waterham et al. 2016). Authored: May, Bruce, 2017-12-15 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-15 LEFT-TO-RIGHT PEX5S,L binds cargo proteins containing PTS1 It is unclear how the long isoform of PEX5 (PEX5L) and the short isoform of PEX5 (PEX5S) are generated. A current hypothesis suggests alternative mRNA splicing. Both isoforms can bind the peroxisome targeting signal 1 (PTS1) located at the C-terminus of most of the proteins that are targeted to the peroxisomal matrix (Dodt et al. 1995, Fransen et al. 1995, Wiemer et al. 1995, Gatto et al. 2000, Brocard et al. 2003, Gatto et al. 2003, Harper et al. 2003, Ghosh and Berg 2010, Freitas et al. 2011, Okumoto et al. 2011). PTS1 typically contains Ser-Lys-Leu (SKL) at the C-terminus but substantial variation in sequences and affinities for PEX5 are observed and upstream residues can modulate binding to PEX5 (Lametschwandtner et al. 1998, Ghosh and Berg 2010, reviewed in Brocard and Hartig 2006).
A minority of peroxisomal matrix proteins contain PTS2. While the PEX5S isoform binds proteins containing PTS1, the PEX5L isoform binds either proteins containing PTS1 or PEX7 bound to proteins containing PTS2 (Braverman et al. 1998). Some proteins appear to be imported as oligomers, however this is rather inefficient as PEX5 appears to have a preference for monomeric substrates (Otera and Fujiki 2012, Freitas et al. 2011, Freitas et al. 2015, also inferred from mouse homologs). Mutations in PEX5 cause defects in peroxisomal import and comprise complementation group 2 of peroxisomal biogenesis disorders (also called Zellweger spectrum disorders) (Dodt et al. 1995, Wiemer et al. 1995). A specific mutation affecting only the PEX5L isoform is the cause of rhizomelic chondrodysplasia punctate type 5 (Barøy et al. 2015). Authored: May, Bruce, 2017-12-15 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-15 Converted from EntitySet in Reactome PEX5S,L Reactome DB_ID: 9033603 PEX5_HUMAN L PEX5对碘氧基苯甲醚 Peroxisomal targeting signal 1 receptor Reactome DB_ID: 9033103 cytosol GENE ONTOLOGY GO:0005829 UniProt:P50542-1 PEX5 PEX5 PXR1 函数绑定到c端PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import.SUBUNIT Interacts with PEX7 and PEX13 (By similarity). Interacts with PEX12 and PEX14. Interacts (Cys-linked ubiquitinated) with ZFAND6. Interacts with VWA8 in a PEX7-dependent manner (By similarity).TISSUE SPECIFICITY Detected in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.PTM Monoubiquitination at Cys-11 is required for proper export from peroxisomes and recycling.SIMILARITY Belongs to the peroxisomal targeting signal receptor family. Homo sapiens NCBI Taxonomy 9606 UniProt Isoform P50542-1 1 EQUAL 639 EQUAL ReactomeDatabase ID Release 77 9033103 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033103 Reactome R-HSA-9033103 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033103.1 Reactome //www.joaskin.com PEX5_HUMAN PEX5 isoform S Peroxisomal targeting signal 1 receptor PEX5(1-214,252-639) Reactome DB_ID: 9033154 UniProt:P50542-2 PEX5 PEX5 PXR1 函数绑定到c端PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import.SUBUNIT Interacts with PEX7 and PEX13 (By similarity). Interacts with PEX12 and PEX14. Interacts (Cys-linked ubiquitinated) with ZFAND6. Interacts with VWA8 in a PEX7-dependent manner (By similarity).TISSUE SPECIFICITY Detected in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas.PTM Monoubiquitination at Cys-11 is required for proper export from peroxisomes and recycling.SIMILARITY Belongs to the peroxisomal targeting signal receptor family. UniProt Isoform P50542-2 1 EQUAL 639 EQUAL ReactomeDatabase ID Release 77 9033154 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033154 Reactome R-HSA-9033154 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033154.1 ReactomeDatabase ID Release 77 9033603 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033603 Reactome R-HSA-9033603 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033603.1 Converted from EntitySet in Reactome Cargo proteins containing PTS1 Reactome DB_ID: 9033111 ACOX1 Peroxisomal acyl-coenzyme A oxidase 1 ACOX1_HUMAN Reactome DB_ID: 9033085 UniProt:Q15067 ACOX1 ACOX1 ACOX FUNCTION Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. First enzyme of the fatty acid beta-oxidation pathway.PATHWAY Lipid metabolism; peroxisomal fatty acid beta-oxidation.SUBUNIT Homodimer (PubMed:32169171). Interacts with LONP2 (PubMed:18281296).TISSUE SPECIFICITY Widely expressed with highest levels of isoform 1 and isoform 2 detected in testis. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 levels are higher in brain, lung, muscle, white adipose tissue and testis. Levels are almost equal in heart.MISCELLANEOUS Isoform 1 and isoform 2 can reverse the Acox1 null phenotype in mouse which is characterized by severe microvesicular hepatic steatosis, sustained activation of PPARA, spontaneous massive peroxisome proliferation and eventual development of hepatocellular carcinomas. Isoform 2 is more effective in reversal of the phenotype than isoform 1 (PubMed:20195242).SIMILARITY Belongs to the acyl-CoA oxidase family. UniProt Q15067 1 EQUAL 660 EQUAL ReactomeDatabase ID Release 77 9033085 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033085 Reactome R-HSA-9033085 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033085.1 ACOX2 Peroxisomal acyl-coenzyme A oxidase 2 ecNumber1.17.99.3/ecNumber ACOX2_HUMAN Reactome DB_ID: 9033151 UniProt:Q99424 ACOX2 ACOX2 FUNCTION Oxidizes the CoA esters of the bile acid intermediates di- and tri-hydroxycholestanoic acids (PubMed:27884763). Capable of oxidizing short as well as long chain 2-methyl branched fatty acids (By similarity).SUBUNIT Homodimer.TISSUE SPECIFICITY Present in all tissues tested: heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. Most abundant in heart, liver and kidney.SIMILARITY Belongs to the acyl-CoA oxidase family. UniProt Q99424 1 EQUAL 681 EQUAL ReactomeDatabase ID Release 77 9033151 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033151 Reactome R-HSA-9033151 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033151.1 ACOX3 Peroxisomal acyl-coenzyme A oxidase 3 ecNumber1.3.3.6/ecNumber ACOX3_HUMAN Reactome DB_ID: 9033087 UniProt:O15254 ACOX3 ACOX3 BRCOX PRCOX FUNCTION Oxidizes the CoA-esters of 2-methyl-branched fatty acids.PATHWAY Lipid metabolism; peroxisomal fatty acid beta-oxidation.SIMILARITY Belongs to the acyl-CoA oxidase family. UniProt O15254 2 EQUAL 700 EQUAL ReactomeDatabase ID Release 77 9033087 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033087 Reactome R-HSA-9033087 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033087.1 EHHADH Peroxisomal bifunctional enzyme ECHP_HUMAN Reactome DB_ID: 9033147 UniProt:Q08426 EHHADH EHHADH ECHD FUNCTION Peroxisomal trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities. Catalyzes two of the four reactions of the long straight chain fatty acids peroxisomal beta-oxidation pathway. Optimal isomerase for 2,5 double bonds into 3,5 form isomerization in a range of enoyl-CoA species (Probable). Also able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species (By similarity). With HSD17B4, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity (PubMed:15060085). Regulates the amount of medium-chain dicarboxylic fatty acids which are essential regulators of all fatty acid oxidation pathways (By similarity). Also involved in the degradation of long-chain dicarboxylic acids through peroxisomal beta-oxidation (PubMed:15060085).ACTIVITY REGULATION Enzyme activity enhanced by acetylation.PATHWAY Lipid metabolism; fatty acid beta-oxidation.SUBUNIT Monomer.TISSUE SPECIFICITY Liver and kidney. Strongly expressed in the terminal segments of the proximal tubule. Lower amounts seen in the brain.PTM Acetylated, leading to enhanced enzyme activity. Acetylation is enhanced by up to 80% after treatment either with trichostin A (TSA) or with nicotinamide (NAM) with highest increase on Lys-346. Acetylation and enzyme activity increased by about 1.5% on addition of fatty acids.MISCELLANEOUS Absent in patients suffering with peroxisomal disorders such as Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease.SIMILARITY In the N-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.SIMILARITY In the C-terminal section; belongs to the 3-hydroxyacyl-CoA dehydrogenase family. UniProt Q08426 1 EQUAL 723 EQUAL ReactomeDatabase ID Release 77 9033147 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033147 Reactome R-HSA-9033147 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033147.1 HSD17B4 Peroxisomal multifunctional enzyme type 2 DHB4_HUMAN Reactome DB_ID: 9033102 UniProt:P51659 HSD17B4 HSD17B4 EDH17B4 SDR8C1 FUNCTION Bifunctional enzyme acting on the peroxisomal beta-oxidation pathway for fatty acids. Catalyzes the formation of 3-ketoacyl-CoA intermediates from straight-chain, 2-methyl-branched-chain fatty acids bile acid intermediates. With EHHADH, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity (PubMed:10671535).PATHWAY Lipid metabolism; fatty acid beta-oxidation.SUBUNIT Homodimer.TISSUE SPECIFICITY Present in many tissues with highest concentrations in liver, heart, prostate and testis.MISCELLANEOUS The protein is found both as a full-length peptide and in a cleaved version.SIMILARITY Belongs to the short-chain dehydrogenases/reductases (SDR) family. UniProt P51659 1 EQUAL 736 EQUAL ReactomeDatabase ID Release 77 9033102 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033102 Reactome R-HSA-9033102 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033102.1 SCP2 Non-specific lipid-transfer protein NLTP_HUMAN Reactome DB_ID: 9033130 UniProt:P22307 SCP2 SCP2 TISSUE SPECIFICITY Liver, fibroblasts, and placenta.INDUCTION Up-regulated by 4-hydroxy-tamoxifen.DISEASE Expression at protein level is almost abolished in Zellweger syndrome. Cholesterol transfer from the endoplasmic reticulum to the plasma membrane was reduced in patient fibroblasts compared to controls.SIMILARITY In the N-terminal section; belongs to the thiolase-like superfamily. Thiolase family. UniProt P22307 1 EQUAL 547 EQUAL ReactomeDatabase ID Release 77 9033130 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033130 Reactome R-HSA-9033130 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033130.1 ECI2_HUMAN ECI2(39-394) ECI2 isoform 2 Enoyl-CoA delta isomerase 2, mitochondrial ecNumber5.3.3.8/ecNumber Reactome DB_ID: 9033094 UniProt:O75521-2 ECI2 ECI2 DRS1 HCA88 PECI FUNCTION Able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species. Has a preference for 3-trans substrates.PATHWAY Lipid metabolism; fatty acid beta-oxidation.TISSUE SPECIFICITY Abundant in heart, skeletal muscle and liver. Expressed in CD34(+) T-cells and CD34(+) bone marrow cells.SIMILARITY In the C-terminal section; belongs to the enoyl-CoA hydratase/isomerase family.CAUTION It is uncertain whether Met-1 or Met-3 is the initiator. UniProt Isoform O75521-2 39 EQUAL 394 EQUAL ReactomeDatabase ID Release 77 9033094 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033094 Reactome R-HSA-9033094 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033094.1 DECR2 Peroxisomal 2,4-dienoyl-CoA reductase DECR2_HUMAN Reactome DB_ID: 9033149 UniProt:Q9NUI1 DECR2 DECR2 PDCR SDR17C1 FUNCTION Auxiliary enzyme of beta-oxidation. Participates in the degradation of unsaturated fatty enoyl-CoA esters having double bonds in both even- and odd-numbered positions in peroxisome. Catalyzes the NADP-dependent reduction of 2,4-dienoyl-CoA to yield trans-3-enoyl-CoA. Has activity towards short and medium chain 2,4-dienoyl-CoAs, but also towards 2,4,7,10,13,16,19-docosaheptaenoyl-CoA, suggesting that it does not constitute a rate limiting step in the peroxisomal degradation of docosahexaenoic acid.SUBUNIT Monomer, dimer and oligomer.SIMILARITY Belongs to the short-chain dehydrogenases/reductases (SDR) family. 2,4-dienoyl-CoA reductase subfamily. UniProt Q9NUI1 2 EQUAL 292 EQUAL ReactomeDatabase ID Release 77 9033149 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033149 Reactome R-HSA-9033149 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033149.1 Delta ECH1(1-328) ECH1_HUMAN Reactome DB_ID: 9033152 UniProt:Q13011 ECH1 ECH1 FUNCTION Isomerization of 3-trans,5-cis-dienoyl-CoA to 2-trans,4-trans-dienoyl-CoA.PATHWAY Lipid metabolism; fatty acid beta-oxidation.SUBUNIT Homohexamer.SIMILARITY Belongs to the enoyl-CoA hydratase/isomerase family. UniProt Q13011 1 EQUAL 328 EQUAL ReactomeDatabase ID Release 77 9033152 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033152 Reactome R-HSA-9033152 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033152.1 PECR Peroxisomal trans-2-enoyl-CoA reductase PECR_HUMAN Reactome DB_ID: 9033155 UniProt:Q9BY49 PECR PECR SDR29C1 PRO1004 功能参与脂肪链伸长acids. Catalyzes the reduction of trans-2-enoyl-CoAs of varying chain lengths from 6:1 to 16:1, having maximum activity with 10:1 CoA. Has no 2,4-dienoyl-CoA reductase activity.PATHWAY Lipid metabolism; fatty acid biosynthesis.SUBUNIT Interacts with PEX5, probably required to target it into peroxisomes.INDUCTION Not induced by IR.SIMILARITY Belongs to the short-chain dehydrogenases/reductases (SDR) family. UniProt Q9BY49 1 EQUAL 303 EQUAL ReactomeDatabase ID Release 77 9033155 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033155 Reactome R-HSA-9033155 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033155.1 HACL1 2-hydroxyacyl-CoA lyase 1 ecNumber4.1.-.-/ecNumber HACL1_HUMAN Reactome DB_ID: 9033120 UniProt:Q9UJ83 HACL1 HACL1 HPCL HPCL2 PHYH2 HSPC279 FUNCTION Peroxisomal 2-OH acyl-CoA lyase involved in the cleavage (C1 removal) reaction in the fatty acid alpha-oxydation in a thiamine pyrophosphate (TPP)-dependent manner (PubMed:28289220, PubMed:21708296, PubMed:10468558). Involved in the degradation of 3-methyl-branched fatty acids like phytanic acid and the shortening of 2-hydroxy long-chain fatty acids (PubMed:28289220, PubMed:21708296, PubMed:10468558). Plays a significant role in the biosynthesis of heptadecanal in the liver (By similarity).PATHWAY Lipid metabolism; fatty acid metabolism.SUBUNIT Homotetramer.TISSUE SPECIFICITY Widely expressed.SIMILARITY Belongs to the TPP enzyme family. UniProt Q9UJ83 1 EQUAL 578 EQUAL ReactomeDatabase ID Release 77 9033120 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033120 Reactome R-HSA-9033120 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033120.1 AMACR Alpha-methylacyl-CoA racemase ecNumber5.1.99.4/ecNumber AMACR_HUMAN Reactome DB_ID: 9033113 UniProt:Q9UHK6 AMACR AMACR FUNCTION Catalyzes the interconversion of (R)- and (S)-stereoisomers of alpha-methyl-branched-chain fatty acyl-CoA esters (PubMed:7649182, PubMed:10655068, PubMed:11060359). Acts only on coenzyme A thioesters, not on free fatty acids, and accepts as substrates a wide range of alpha-methylacyl-CoAs, including pristanoyl-CoA, trihydroxycoprostanoyl-CoA (an intermediate in bile acid synthesis), and arylpropionic acids like the anti-inflammatory drug ibuprofen (2-(4-isobutylphenyl)propionic acid) but neither 3-methyl-branched nor linear-chain acyl-CoAs (PubMed:7649182, PubMed:10655068, PubMed:11060359).PATHWAY Lipid metabolism; bile acid biosynthesis.PATHWAY Lipid metabolism; fatty acid metabolism.SUBUNIT Monomer.SIMILARITY Belongs to the CoA-transferase III family. UniProt Q9UHK6 1 EQUAL 382 EQUAL ReactomeDatabase ID Release 77 9033113 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033113 Reactome R-HSA-9033113 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033113.1 CRAT Carnitine O-acetyltransferase CACP_HUMAN Reactome DB_ID: 9033096 UniProt:P43155 CRAT CRAT CAT1 FUNCTION Catalyzes the reversible transfer of acyl groups from carnitine to coenzyme A (CoA) and regulates the acyl-CoA/CoA ratio. Also plays a crucial role in the transport of fatty acids for beta-oxidation (PubMed:15099582, PubMed:29395073). Responsible for the synthesis of short- and branched-chain acylcarnitines (PubMed:23485643). Active towards some branched-chain amino acid oxidation pathway (BCAAO) intermediates (PubMed:23485643). Trans-2-enoyl-CoAs and 2-methylacyl-CoAs are poor substrates (PubMed:23485643).SUBUNIT Monomer.TISSUE SPECIFICITY Mostly in skeletal muscle, less in heart, liver and pancreas, only weakly detectable in brain, placenta, lung and kidney.SIMILARITY Belongs to the carnitine/choline acetyltransferase family. UniProt P43155 1 EQUAL 626 EQUAL ReactomeDatabase ID Release 77 9033096 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033096 Reactome R-HSA-9033096 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033096.1 CROT Peroxisomal carnitine O-octanoyltransferase OCTC_HUMAN Reactome DB_ID: 9033106 UniProt: Q9UKG9 CROT CROT COT FUNCTION Beta-oxidation of fatty acids. The highest activity concerns the C6 to C10 chain length substrate. Converts the end product of pristanic acid beta oxidation, 4,8-dimethylnonanoyl-CoA, to its corresponding carnitine ester.PATHWAY Lipid metabolism; fatty acid beta-oxidation.SUBUNIT Monomer.SIMILARITY Belongs to the carnitine/choline acetyltransferase family. UniProt Q9UKG9 1 EQUAL 612 EQUAL ReactomeDatabase ID Release 77 9033106 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033106 Reactome R-HSA-9033106 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033106.1 ACOT8 Acyl-coenzyme A thioesterase 8 ACOT8_HUMAN Reactome DB_ID: 9033134 UniProt:O14734 ACOT8 ACOT8 ACTEIII PTE1 函数Acyl-coenzyme thioesterases(酰coa)are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH (PubMed:9299485, PubMed:9153233, PubMed:15194431). Acyl-coenzyme A thioesterase 8/ACOT8 display no strong substrate specificity with respect to the carboxylic acid moiety of Acyl-CoAs (By similarity). Hydrolyzes medium length (C2 to C20) straight-chain, saturated and unsaturated acyl-CoAS but is inactive towards substrates with longer aliphatic chains (PubMed:9299485, PubMed:9153233). Moreover, it catalyzes the hydrolysis of CoA esters of bile acids, such as choloyl-CoA and chenodeoxycholoyl-CoA and competes with bile acid CoA:amino acid N-acyltransferase (BAAT) (By similarity). ACOT8 is also able to hydrolyze CoA esters of dicarboxylic acids (By similarity). It is involved in the metabolic regulation of peroxisome proliferation (PubMed:15194431).FUNCTION (Microbial infection) May mediate Nef-induced down-regulation of CD4 cell-surface expression (PubMed:9153233).ACTIVITY REGULATION Inhibited by CoASH (IC(50)=10-15 uM). Also inhibited by cysteine-reactive agents.PATHWAY Lipid metabolism; fatty acid metabolism.SUBUNIT Homodimer (By similarity).SUBUNIT (Microbial infection) Interacts with human immunodeficiency virus (HIV-1) Nef (via middle region); this interaction enhances ACOT8 Acyl-CoA thioesterase activity and occurs in a Nef myristoylation-independent manner (PubMed:9299485). According to a second report, the interaction with HIV-1 Nef occurs in a Nef myristoylation-independent manner but does not enhance ACOT8 Acyl-CoA thioesterase activity (PubMed:9153233).TISSUE SPECIFICITY Detected in a T-cell line (at protein level). Ubiquitous (PubMed:9153233, PubMed:9299485).INDUCTION Regulated by peroxisome proliferator (such as Clofibrate), via the peroxisome proliferator-activated receptors (PPARs).SIMILARITY Belongs to the C/M/P thioester hydrolase family. UniProt O14734 1 EQUAL 319 EQUAL ReactomeDatabase ID Release 77 9033134 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033134 Reactome R-HSA-9033134 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033134.1 ACOT2_HUMAN ACOT2(1-483) Acyl-coenzyme A thioesterase 2, mitochondrial Reactome DB_ID: 9033137 UniProt:P49753 ACOT2 ACOT2 PTE2 PTE2A FUNCTION Acyl-CoA thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH (PubMed:16940157). Acyl-coenzyme A thioesterase 2/ACOT2 displays higher activity toward long chain acyl CoAs (C14-C20) (PubMed:16940157, PubMed:10944470). The enzyme is involved in enhancing the hepatic fatty acid oxidation in mitochondria (By similarity).PATHWAY Lipid metabolism; fatty acid metabolism.SUBUNIT Monomer.TISSUE SPECIFICITY Strongest expression in heart, liver, muscle and kidney. Weak in placenta and pancreas.SIMILARITY Belongs to the C/M/P thioester hydrolase family.CAUTION Was originally (PubMed:10944470) thought to be peroxisomal but was later shown (PubMed:16940157) to be mitochondrial. UniProt P49753 1 EQUAL 483 EQUAL ReactomeDatabase ID Release 77 9033137 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033137 Reactome R-HSA-9033137 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033137.1 CAT Catalase ecNumber1.11.1.6/ecNumber CATA_HUMAN Reactome DB_ID: 9033141 UniProt:P04040 CAT CAT FUNCTION Occurs in almost all aerobically respiring organisms and serves to protect cells from the toxic effects of hydrogen peroxide. Promotes growth of cells including T-cells, B-cells, myeloid leukemia cells, melanoma cells, mastocytoma cells and normal and transformed fibroblast cells.SUBUNIT Homotetramer.PTM The N-terminus is blocked.SIMILARITY Belongs to the catalase family. UniProt P04040 2 EQUAL 527 EQUAL ReactomeDatabase ID Release 77 9033141 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033141 Reactome R-HSA-9033141 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033141.1 DAO D-amino-acid oxidase OXDA_HUMAN Reactome DB_ID: 9033150 UniProt:P14920 DAO DAO DAMOX 功能调节神经调节物质的水平D-serine in the brain. Has high activity towards D-DOPA and contributes to dopamine synthesis. Could act as a detoxifying agent which removes D-amino acids accumulated during aging. Acts on a variety of D-amino acids with a preference for those having small hydrophobic side chains followed by those bearing polar, aromatic, and basic groups. Does not act on acidic amino acids.SUBUNIT Homodimer (PubMed:17088322, PubMed:18455394). Interacts with DAOA (PubMed:12364586).SIMILARITY Belongs to the DAMOX/DASOX family. UniProt P14920 1 EQUAL 347 EQUAL ReactomeDatabase ID Release 77 9033150 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033150 Reactome R-HSA-9033150 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033150.1 DDO D-aspartate oxidase OXDD_HUMAN Reactome DB_ID: 9033121 UniProt:Q99489 DDO DDO FUNCTION Selectively catalyzes the oxidative deamination of D-aspartate and its N-methylated derivative, N-methyl D-aspartate.SIMILARITY Belongs to the DAMOX/DASOX family. UniProt Q99489 1 EQUAL 341 EQUAL ReactomeDatabase ID Release 77 9033121 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033121 Reactome R-HSA-9033121 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033121.1 HAO1 Hydroxyacid oxidase 1 HAOX1_HUMAN Reactome DB_ID: 9033108 UniProt:Q9UJM8 HAO1 HAO1 GOX1 HAOX1 FUNCTION Has 2-hydroxyacid oxidase activity. Most active on the 2-carbon substrate glycolate, but is also active on 2-hydroxy fatty acids, with high activity towards 2-hydroxy palmitate and 2-hydroxy octanoate.PATHWAY Organic acid metabolism; glycolate degradation; 3-phospho-D-glycerate from glycolate: step 1/4.TISSUE SPECIFICITY Liver.SIMILARITY Belongs to the FMN-dependent alpha-hydroxy acid dehydrogenase family. UniProt Q9UJM8 1 EQUAL 370 EQUAL ReactomeDatabase ID Release 77 9033108 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033108 Reactome R-HSA-9033108 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033108.1 HAO2 Hydroxyacid oxidase 2 HAOX2_HUMAN Reactome DB_ID: 9033093 UniProt:Q9NYQ3 HAO2 HAO2 HAOX2 GIG16 FUNCTION Catalyzes the oxidation of L-alpha-hydroxy acids as well as, more slowly, that of L-alpha-amino acids.SUBUNIT Homotetramer or homooctamer.TISSUE SPECIFICITY Liver and kidney.SIMILARITY Belongs to the FMN-dependent alpha-hydroxy acid dehydrogenase family. UniProt Q9NYQ3 1 EQUAL 351 EQUAL ReactomeDatabase ID Release 77 9033093 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033093 Reactome R-HSA-9033093 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033093.1 SLC27A2 Very long-chain acyl-CoA synthetase S27A2_HUMAN Reactome DB_ID: 9033090 UniProt:O14975 SLC27A2 SLC27A2 ACSVL1 FACVL1 FATP2 VLACS FUNCTION Acyl CoA synthetase that activates long-chain and very long-chain fatty acids (VLCFAs) by catalyzing the formation of fatty acyl-CoA (PubMed:10198260, PubMed:10749848, PubMed:11980911). Can also activate branched-chain fatty acids such as phytanic acid and pristanic acid (PubMed:10198260). Does not activate C24 bile acids, cholate and chenodeoxycholate (PubMed:11980911). In vitro, activates 3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanate (THCA), the C27 precursor of cholic acid deriving from the de novo synthesis from cholesterol (PubMed:11980911). Exhibits long-chain fatty acids (LCFA) transport activity and plays an important role in hepatic fatty acid uptake (PubMed:20530735).TISSUE SPECIFICITY Expressed in liver, kidney, placenta and pancreas.SIMILARITY Belongs to the ATP-dependent AMP-binding enzyme family. UniProt O14975 1 EQUAL 620 EQUAL ReactomeDatabase ID Release 77 9033090 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033090 Reactome R-HSA-9033090 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033090.1 GNPAT Dihydroxyacetone phosphate acyltransferase GNPAT_HUMAN Reactome DB_ID: 9033129 UniProt:O15228 GNPAT GNPAT DAPAT DHAPAT FUNCTION Dihydroxyacetonephosphate acyltransferase involved in plasmalogen biosynthesis.PATHWAY Membrane lipid metabolism; glycerophospholipid metabolism.SUBUNIT May be part of a heterotrimeric complex composed of DAP-AT, ADAP-S and a modified form of DAP-AT.DOMAIN The HXXXXD motif is essential for acyltransferase activity and may constitute the binding site for the phosphate moiety of the glycerol-3-phosphate.SIMILARITY Belongs to the GPAT/DAPAT family. UniProt O15228 1 EQUAL 680 EQUAL ReactomeDatabase ID Release 77 9033129 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033129 Reactome R-HSA-9033129 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033129.1 IDH1 Isocitrate dehydrogenase (NADP) cytoplasmic PICD ReactomeDB_ID: 389549 UniProt:O75874 IDH1 IDH1 PICD SUBUNIT Homodimer.PTM Acetylation at Lys-374 dramatically reduces catalytic activity.DISEASE Genetic variations are associated with cartilaginous tumors such as enchondroma or chondrosarcoma. Mutations of Arg-132 to Cys, Gly or His abolish the conversion of isocitrate to alpha-ketoglutarate. Instead, alpha-ketoglutarate is converted to R(-)-2-hydroxyglutarate.SIMILARITY Belongs to the isocitrate and isopropylmalate dehydrogenases family. UniProt O75874 1 EQUAL 414 EQUAL ReactomeDatabase ID Release 77 389549 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=389549 Reactome R-HSA-389549 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-389549.1 AGXT Serine--pyruvate aminotransferase SPYA_HUMAN Reactome DB_ID: 9033128 UniProt:P21549 AGXT AGXT AGT1 SPAT SUBUNIT Homodimer.TISSUE SPECIFICITY Liver.POLYMORPHISM Polymorphism at position 11 acts synergistically with different mutations in AGXT producing specific enzymatic phenotypes in HP1 patients. The combined presence of Leu-11 and Met-340 polymorphisms defines the minor AGXT allele, whereas their absence defines the major allele. The minor allele has frequencies of 20% in normal European and North American populations, and 50% in HP1 patients.SIMILARITY Belongs to the class-V pyridoxal-phosphate-dependent aminotransferase family. UniProt P21549 1 EQUAL 392 EQUAL ReactomeDatabase ID Release 77 9033128 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033128 Reactome R-HSA-9033128 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033128.1 PIPOX Peroxisomal sarcosine oxidase SOX_HUMAN Reactome DB_ID: 9033136 UniProt:Q9P0Z9 PIPOX PIPOX LPIPOX PSO 功能代谢肌氨酸,L-pipecolic酸nd L-proline.SUBUNIT Monomer.SIMILARITY Belongs to the MSOX/MTOX family. UniProt Q9P0Z9 1 EQUAL 390 EQUAL ReactomeDatabase ID Release 77 9033136 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033136 Reactome R-HSA-9033136 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033136.1 EPHX2 Epoxide hydrolase 2 HYES_HUMAN ReactomeDB_ID: 2142819 UniProt:P34913 EPHX2 EPHX2 FUNCTION Bifunctional enzyme (PubMed:12574510). The C-terminal domain has epoxide hydrolase activity and acts on epoxides (alkene oxides, oxiranes) and arene oxides (PubMed:12869654, PubMed:12574510, PubMed:22798687). Plays a role in xenobiotic metabolism by degrading potentially toxic epoxides (By similarity). Also determines steady-state levels of physiological mediators (PubMed:12869654, PubMed:12574510, PubMed:22798687, PubMed:21217101).FUNCTION Bifunctional enzyme (PubMed:12574510). The N-terminal domain has lipid phosphatase activity, with the highest activity towards threo-9,10-phosphonooxy-hydroxy-octadecanoic acid, followed by erythro-9,10-phosphonooxy-hydroxy-octadecanoic acid, 12-phosphonooxy-octadec-9Z-enoic acid and 12-phosphonooxy-octadec-9E-enoic acid (PubMed:12574510). Has phosphatase activity toward lyso-glycerophospholipids with also some lower activity toward lysolipids of sphingolipid and isoprenoid phosphates (PubMed:22217705, PubMed:22387545).ACTIVITY REGULATION Inhibited by 1-(1-acetylpiperidin-4-yl)-3-(4-(trifl uoromethoxy)phenyl)urea (TPAU), 1-cyclohexyl-3-dodecylurea (CDU), 12-(3-adamantan-1-yl-ureido)-dodecanoic acid (AUDA), 1-((3S, 5S, 7S)-adamantan-1-yl)-3-(5-(2-(2-ethoxyethoxy) ethoxy)pentyl)urea (AEPU), N-adamantyl-N[']-cyclohexyl urea (ACU), 4-(((1S, 4S)-4-(3-((3S, 5S, 7S)-adamantan-1-yl) ureido)cyclohexyl)oxy)benzoic acid (c-AUCB), 4-(((1R, 4R)-4-(3-((3S, 5S, 7S)-adamantan-1-yl)ureido)cyclohexyl)oxy)benzoic acid (t-AUCB), 4-(((1R, 4R)-4-(3-(4(trifluoromethoxy)phenyl)ureido)cyclohexyl)oxy)benzoic acid (t-TAUCB) and to a lesser extent by 8-(3-((3S, 5S, 7S)-adamantan-1-yl)ureido) octanoic acid (AUOA). Phosphatase activity is inhibited by dodecyl-phosphate, phospholipids such as phospho-lysophosphatidic acids and fatty acids such as palmitic acid and lauric acid (PubMed:22217705, PubMed:22387545).SUBUNIT Homodimer.INDUCTION By compounds that cause peroxisome proliferation such as clofibrate, tiadenol and fenofibrate.DOMAIN The N-terminal domain has phosphatase activity. The C-terminal domain has epoxide hydrolase activity.PTM The N-terminus is blocked.PTM The covalent modification of cysteine by 15-deoxy-Delta12,14-prostaglandin-J2 is autocatalytic and reversible. It may occur as an alternative to other cysteine modifications, such as S-nitrosylation and S-palmitoylation (Probable).SIMILARITY Belongs to the AB hydrolase superfamily. Epoxide hydrolase family. UniProt P34913 1 EQUAL 555 EQUAL ReactomeDatabase ID Release 77 2142819 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=2142819 Reactome R-HSA-2142819 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-2142819.1 GSTK1 Glutathione S-transferase kappa 1 ReactomeDB_ID: 176078 UniProt:Q9Y2Q3 GSTK1 GSTK1 HDCMD47P FUNCTION Significant glutathione conjugating activity is found only with the model substrate, 1-chloro-2,4-dinitrobenzene (CDNB).SUBUNIT Homodimer.TISSUE SPECIFICITY Ubiquitous.SIMILARITY Belongs to the GST superfamily. Kappa family. UniProt Q9Y2Q3 2 EQUAL 226 EQUAL ReactomeDatabase ID Release 77 176078 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=176078 Reactome R-HSA-176078 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-176078.1 PAOX Peroxisomal N PAOX_HUMAN Reactome DB_ID: 9033109 UniProt:Q6QHF9 PAOX PAOX PAO UNQ1923/PRO4398 FUNCTION Flavoenzyme which catalyzes the oxidation of N(1)-acetylspermine to spermidine and is thus involved in the polyamine back-conversion (PubMed:12477380). Can also oxidize N(1)-acetylspermidine to putrescine. Substrate specificity: N(1)-acetylspermine = N(1)-acetylspermidine > N(1),N(12)-diacylspermine >> spermine. Does not oxidize spermidine. Plays an important role in the regulation of polyamine intracellular concentration and has the potential to act as a determinant of cellular sensitivity to the antitumor polyamine analogs (PubMed:12477380).PATHWAY Amine and polyamine metabolism; spermine metabolism.SUBUNIT Monomer.TISSUE SPECIFICITY Widely expressed. Not detected in spleen. Expressed at lower level in neoplastic tissues.INDUCTION By polyamine analogs.MISCELLANEOUS Oxidizes N(1)-acetylated polyamines on the exo-side of their N(4)-amino groups. Plant PAO oxidizes spermine on the endo-side of the N(4)-nitrogen (By similarity).SIMILARITY Belongs to the flavin monoamine oxidase family. UniProt Q6QHF9 1 EQUAL 649 EQUAL ReactomeDatabase ID Release 77 9033109 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033109 Reactome R-HSA-9033109 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033109.1 DCMC_HUMAN MLYCD(40-493) Malonyl-CoA decarboxylase, mitochondrial Reactome DB_ID: 9033089 UniProt:O95822-2 MLYCD MLYCD FUNCTION Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced. In peroxisomes it may be involved in degrading intraperoxisomal malonyl-CoA, which is generated by the peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids. Plays a role in the metabolic balance between glucose and lipid oxidation in muscle independent of alterations in insulin signaling. May play a role in controlling the extent of ischemic injury by promoting glucose oxidation.ACTIVITY REGULATION Malonyl-CoA decarboxylase activity does not require any cofactors or divalent metal ions. Formation of interchain disulfide bonds leads to positive cooperativity between active sites and increases the affinity for malonyl-CoA and the catalytic efficiency (in vitro).PATHWAY Metabolic intermediate biosynthesis; acetyl-CoA biosynthesis; acetyl-CoA from malonyl-CoA: step 1/1.SUBUNIT Homotetramer. Dimer of dimers. The two subunits within a dimer display conformational differences suggesting that at any given moment, only one of the two subunits is competent for malonyl-CoA binding and catalytic activity. Under oxidizing conditions, can form disulfide-linked homotetramers (in vitro). Associates with the peroxisomal targeting signal receptor PEX5.TISSUE SPECIFICITY Expressed in fibroblasts and hepatoblastoma cells (at protein level). Expressed strongly in heart, liver, skeletal muscle, kidney and pancreas. Expressed in myotubes. Expressed weakly in brain, placenta, spleen, thymus, testis, ovary and small intestine.PTM Acetylation at Lys-472 activates malonyl-CoA decarboxylase activity. Deacetylation at Lys-472 by SIRT4 represses activity, leading to promote lipogenesis (By similarity).PTM Interchain disulfide bonds may form in peroxisomes (Potential). Interchain disulfide bonds are not expected to form in the reducing environment of the cytoplasm and mitochondria. UniProt Isoform O95822-2 40 EQUAL 493 EQUAL ReactomeDatabase ID Release 77 9033089 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033089 Reactome R-HSA-9033089 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033089.1 HMGCL_HUMAN HMGCL(1-325) Hydroxymethylglutaryl-CoA lyase, mitochondrial Reactome DB_ID: 9033112 UniProt:P35914 HMGCL HMGCL FUNCTION Mitochondrial 3-hydroxymethyl-3-methylglutaryl-CoA lyase that catalyzes a cation-dependent cleavage of (S)-3-hydroxy-3-methylglutaryl-CoA into acetyl-CoA and acetoacetate, a key step in ketogenesis. Terminal step in leucine catabolism. Ketone bodies (beta-hydroxybutyrate, acetoacetate and acetone) are essential as an alternative source of energy to glucose, as lipid precursors and as regulators of metabolism.ACTIVITY REGULATION Stimulated by reducing agents such as dithiothreitol (DTT).PATHWAY Metabolic intermediate metabolism; (S)-3-hydroxy-3-methylglutaryl-CoA degradation; acetoacetate from (S)-3-hydroxy-3-methylglutaryl-CoA: step 1/1.SUBUNIT Homodimer; disulfide-linked. Can also form homotetramers.TISSUE SPECIFICITY Highest expression in liver. Expressed in pancreas, kidney, intestine, testis, fibroblasts and lymphoblasts. Very low expression in brain and skeletal muscle. The relative expression of isoform 2 (at mRNA level) is highest in heart (30%), skeletal muscle (22%), and brain (14%).SIMILARITY Belongs to the HMG-CoA lyase family. UniProt P35914 1 EQUAL 325 EQUAL ReactomeDatabase ID Release 77 9033112 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033112 Reactome R-HSA-9033112 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033112.1 NUDT7 Peroxisomal coenzyme A diphosphatase NUDT7 ecNumber3.6.1.-/ecNumber NUDT7_HUMAN Reactome DB_ID: 9033118 UniProt:P0C024 NUDT7 NUDT7 辅酶diphosphatase介导t的函数he cleavage of CoA, CoA esters and oxidized CoA with similar efficiencies, yielding 3',5'-ADP and the corresponding 4'-phosphopantetheine derivative as products. Preferentially hydrolyzes medium-chain acyl-CoAs and bile acid-CoAs. CoA into 3',5'-ADP and 4'-phosphopantetheine. Has no activity toward NDP-sugars, CDP-alcohols, (deoxy)nucleoside 5'-triphosphates, nucleoside 5'-di or monophosphates, diadenosine polyphosphates, NAD, NADH, NADP, NADPH or thymidine-5'-monophospho-p-nitrophenyl ester. May be required to eliminate oxidized CoA from peroxisomes, or regulate CoA and acyl-CoA levels in this organelle in response to metabolic demand. Does not play a role in U8 snoRNA decapping activity. Binds U8 snoRNA.ACTIVITY REGULATION Inhibited by fluoride.TISSUE SPECIFICITY Expressed in liver, kidney, pancreas, pituitary, small intestine, spleen, heart and placenta. Weakly expressed in brain.SIMILARITY Belongs to the Nudix hydrolase family. PCD1 subfamily. UniProt P0C024 1 EQUAL 238 EQUAL ReactomeDatabase ID Release 77 9033118 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033118 Reactome R-HSA-9033118 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033118.1 IDE Insulin-degrading enzyme ecNumber3.4.24.56/ecNumber IDE_HUMAN Reactome DB_ID: 9033133 UniProt:P14735 IDE IDE 函数在细胞分解的过程中发挥作用insulin, APP peptides, IAPP peptides, natriuretic peptides, glucagon, bradykinin, kallidin, and other peptides, and thereby plays a role in intercellular peptide signaling (PubMed:2293021, PubMed:10684867, PubMed:26968463, PubMed:17051221, PubMed:17613531, PubMed:18986166, PubMed:19321446, PubMed:23922390, PubMed:24847884, PubMed:26394692, PubMed:29596046, PubMed:21098034). Substrate binding induces important conformation changes, making it possible to bind and degrade larger substrates, such as insulin (PubMed:23922390, PubMed:26394692, PubMed:29596046). Contributes to the regulation of peptide hormone signaling cascades and regulation of blood glucose homeostasis via its role in the degradation of insulin, glucagon and IAPP (By similarity). Plays a role in the degradation and clearance of APP-derived amyloidogenic peptides that are secreted by neurons and microglia (PubMed:9830016, PubMed:26394692) (Probable). Degrades the natriuretic peptides ANP, BNP and CNP, inactivating their ability to raise intracellular cGMP (PubMed:21098034). Also degrades an aberrant frameshifted 40-residue form of NPPA (fsNPPA) which is associated with familial atrial fibrillation in heterozygous patients (PubMed:21098034). Involved in antigen processing. Produces both the N terminus and the C terminus of MAGEA3-derived antigenic peptide (EVDPIGHLY) that is presented to cytotoxic T lymphocytes by MHC class I.FUNCTION (Microbial infection) The membrane-associated isoform acts as an entry receptor for varicella-zoster virus (VZV).ACTIVITY REGULATION Activated by small peptides (By similarity). Activated by ATP and GTP, and to a lesser extent by CTP, TTP and PPPi (PubMed:17613531). Inhibited by bacitracin (PubMed:17055432, PubMed:17613531). In vitro modification of Cys residues impairs enzyme activity (PubMed:18986166).SUBUNIT Homodimer (PubMed:17051221, PubMed:19321446, PubMed:23922390, PubMed:26394692, PubMed:29596046) (Probable). Can also form homotetramers (By similarity).SUBUNIT (Microbial infection) Interacts (via N-terminus) with varicella-zoster virus (VZV) envelope glycoprotein E (via N-terminus); the membrane-associated isoform may function as an entry receptor for this virus (PubMed:17055432, PubMed:17553876).TISSUE SPECIFICITY Detected in brain and in cerebrospinal fluid (at protein level).DOMAIN The SlyX motif may be involved in the non-conventional secretion of the protein.PTM The N-terminus is blocked.MISCELLANEOUS ATP-binding induces a conformation change.SIMILARITY Belongs to the peptidase M16 family. UniProt P14735 1 EQUAL 1019 EQUAL ReactomeDatabase ID Release 77 9033133 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033133 Reactome R-HSA-9033133 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033133.1 LONP2 ReactomeDB_ID: 8963025 UniProt:Q86WA8 LONP2 LONP2 LONP FUNCTION ATP-dependent serine protease that mediates the selective degradation of misfolded and unassembled polypeptides in the peroxisomal matrix. Necessary for type 2 peroxisome targeting signal (PTS2)-containing protein processing and facilitates peroxisome matrix protein import (By similarity). May indirectly regulate peroxisomal fatty acid beta-oxidation through degradation of the self-processed forms of TYSND1.SUBUNIT Interacts with PEX5 (By similarity) (PubMed:22002062). Interacts with TYSND1 (By similarity) (PubMed:22002062). May interact with enzymes involved in beta-oxidation of fatty acids, including ACOX1/AOX (PubMed:18281296).TISSUE SPECIFICITY Widely expressed, with high levels in the liver, kidney and pancreas.SIMILARITY Belongs to the peptidase S16 family. UniProt Q86WA8 2 EQUAL 852 EQUAL ReactomeDatabase ID Release 77 8963025 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8963025 Reactome R-HSA-8963025 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8963025.1 NUDT19 Nucleoside diphosphate-linked moiety X motif 19 NUD19_HUMAN Reactome DB_ID: 9033098 UniProt:A8MXV4 NUDT19 NUDT19 FUNCTION Coenzyme A diphosphatase that mediates the hydrolysis of a wide range of CoA esters, including choloyl-CoA and branched-chain fatty-acyl-CoA esters. At low substrate concentrations medium and long-chain fatty-acyl-CoA esters are the primary substrates (By similarity).SIMILARITY Belongs to the Nudix hydrolase family. UniProt A8MXV4 1 EQUAL 375 EQUAL ReactomeDatabase ID Release 77 9033098 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033098 Reactome R-HSA-9033098 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033098.1 BAAT Bile acid-CoA:amino acid N-acetyltransferase ReactomeDB_ID: 159392 UniProt:Q14032 BAAT BAAT FUNCTION Catalyzes the amidation of bile acids (BAs) with the amino acids taurine and glycine (PubMed:12810727, PubMed:8034703, PubMed:2037576, PubMed:12239217). More than 95% of the BAs are N-acyl amidates with glycine and taurine (PubMed:8034703). Amidation of BAs in the liver with glycine or taurine prior to their excretion into bile is an important biochemical event in bile acid metabolism (PubMed:12810727). This conjugation (or amidation) plays several important biological roles in that it promotes the secretion of BAs and cholesterol into bile and increases the detergent properties of BAs in the intestine, which facilitates lipid and vitamin absorption (PubMed:12810727). May also act as an acyl-CoA thioesterase that regulates intracellular levels of free fatty acids (PubMed:12810727, PubMed:8034703, PubMed:12239217). In vitro, catalyzes the hydrolysis of long- and very long-chain saturated acyl-CoAs to the free fatty acid and coenzyme A (CoASH), and conjugates glycine to these acyl-CoAs (PubMed:12810727).SUBUNIT Monomer.TISSUE SPECIFICITY Expressed in liver, gallbladder mucosa and pancreas.SIMILARITY Belongs to the C/M/P thioester hydrolase family. UniProt Q14032 1 EQUAL 418 EQUAL ReactomeDatabase ID Release 77 159392 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=159392 Reactome R-HSA-159392 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-159392.1 DHRS4 Dehydrogenase/reductase SDR family member 4 ecNumber1.1.1.184/ecNumber DHRS4_HUMAN Reactome DB_ID: 9033122 UniProt:Q9BTZ2 DHRS4 DHRS4 SDR25C2 UNQ851/PRO1800 FUNCTION Reduces all-trans-retinal and 9-cis retinal. Can also catalyze the oxidation of all-trans-retinol with NADP as co-factor, but with much lower efficiency. Reduces alkyl phenyl ketones and alpha-dicarbonyl compounds with aromatic rings, such as pyrimidine-4-aldehyde, 3-benzoylpyridine, 4-benzoylpyridine, menadione and 4-hexanoylpyridine. Has no activity towards aliphatic aldehydes and ketones (By similarity).SUBUNIT Homotetramer.TISSUE SPECIFICITY Isoform 1 is predominantly expressed in normal cervix (at protein level). Isoform 4 is expressed in some neoplastic cervical tissues, but not in normal cervix (at protein level). Isoform 5 and isoform 6 are expressed in a few neoplastic cervical tissues.MISCELLANEOUS Inhibited by kaempferol, quercetin, genistein and myristic acid.SIMILARITY Belongs to the short-chain dehydrogenases/reductases (SDR) family. UniProt Q9BTZ2 1 EQUAL 278 EQUAL ReactomeDatabase ID Release 77 9033122 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033122 Reactome R-HSA-9033122 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033122.1 NOS2 Nitric oxide synthase, inducible iNOS ReactomeDB_ID: 202107 UniProt:P35228 NOS2 NOS2 NOS2A 函数产生一氧化氮(NO)这是一个市场经济地位senger molecule with diverse functions throughout the body (PubMed:7531687, PubMed:7544004). In macrophages, NO mediates tumoricidal and bactericidal actions. Also has nitrosylase activity and mediates cysteine S-nitrosylation of cytoplasmic target proteins such PTGS2/COX2 (By similarity). As component of the iNOS-S100A8/9 transnitrosylase complex involved in the selective inflammatory stimulus-dependent S-nitrosylation of GAPDH on 'Cys-247' implicated in regulation of the GAIT complex activity and probably multiple targets including ANXA5, EZR, MSN and VIM (PubMed:25417112). Involved in inflammation, enhances the synthesis of proinflammatory mediators such as IL6 and IL8 (PubMed:19688109).ACTIVITY REGULATION Regulated by calcium/calmodulin. Aspirin inhibits expression and function of this enzyme and effects may be exerted at the level of translational/post-translational modification and directly on the catalytic activity (By similarity).SUBUNIT Homodimer (PubMed:10409685, PubMed:10074942). Interacts with SLC9A3R1 (PubMed:12080081). Interacts with GAPDH; induced by oxidatively-modified low-densitity lipoprotein (LDL(ox)) (PubMed:25417112). Interacts with S100A8 and S100A9 to form the iNOS-S100A8/9 transnitrosylase complex (PubMed:25417112). Interacts with SPSB1, SPSB2 and SPSB4 (PubMed:21199876). Interacts with ELOC and CUL5 in the presence of SPSB1 or SPSB2 or SPSB4 (PubMed:21199876).TISSUE SPECIFICITY Expressed in the liver, retina, bone cells and airway epithelial cells of the lung. Not expressed in the platelets.INDUCTION By endotoxins and cytokines. Induced by IFNG/IFN-gamma acting synergistically with bacterial lipopolysaccharides (LPS), TNF or IL1B/interleukin-1 beta (PubMed:7528267). Down-regulated by zinc due to inhibition of NF-kappa-B transactivation activity (PubMed:25180171). By oxidatively-modified low-densitity lipoprotein (LDL(ox)) (PubMed:25417112).PTM Polyubiquitinated; mediated by SPSB1, SPSB2 and SPSB4, leading to proteasomal degradation.POLYMORPHISM Genetic variations in NOS2 are involved in resistance to malaria [MIM:611162].SIMILARITY Belongs to the NOS family. UniProt P35228 1 EQUAL 1153 EQUAL ReactomeDatabase ID Release 77 202107 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=202107 Reactome R-HSA-202107 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-202107.1 MPV17 Protein Mpv17 MPV17_HUMAN Reactome DB_ID: 9033135 UniProt:P39210 MPV17 MPV17 函数的通道的调节membrane potential under normal conditions and oxidative stress, and is involved in mitochondrial homeostasis (PubMed:25861990). Involved in mitochondrial deoxynucleoside triphosphates (dNTP) pool homeostasis and mitochondrial DNA (mtDNA) maintenance (PubMed:26760297). May be involved in the regulation of reactive oxygen species metabolism and the control of oxidative phosphorylation (By similarity).TISSUE SPECIFICITY Ubiquitous. Expressed in pancreas, kidney, muscle, liver, lung, placenta, brain and heart.SIMILARITY Belongs to the peroxisomal membrane protein PXMP2/4 family. UniProt P39210 1 EQUAL 176 EQUAL ReactomeDatabase ID Release 77 9033135 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033135 Reactome R-HSA-9033135 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033135.1 ACOT4 Acyl-coenzyme A thioesterase 4 ACOT4_HUMAN Reactome DB_ID: 9033145 UniProt:Q8N9L9 ACOT4 ACOT4 PTE2B PTEIB FUNCTION Acyl-CoA thioesterases are a group of enzymes that catalyze the hydrolysis of acyl-CoAs to the free fatty acid and coenzyme A (CoASH), providing the potential to regulate intracellular levels of acyl-CoAs, free fatty acids and CoASH (PubMed:16940157). ACOT4 is a peroxisomal succinyl-coenzyme A thioesterase can also hydrolyze glutaryl-CoA and long chain saturated acyl-CoAs (PubMed:16940157).PATHWAY Lipid metabolism; fatty acid biosynthesis.TISSUE SPECIFICITY Strongest expression in liver and kidney and weaker expression in placenta, heart, and muscle.MISCELLANEOUS Compared to mouse peroxisomal succinyl-coenzyme A thioesterase/ACOT4, the human enzyme has a broad substrate specificity overlapping the activity of three mouse acyl-coenzyme A thioesterases, providing an explanation for the unexpectedly low number of acyl-coenzyme A thioesterase genes in the human genome.SIMILARITY Belongs to the C/M/P thioester hydrolase family. UniProt Q8N9L9 1 EQUAL 421 EQUAL ReactomeDatabase ID Release 77 9033145 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033145 Reactome R-HSA-9033145 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033145.1 TYSND1 Peroxisomal leader peptide-processing protease ecNumber3.4.21.-/ecNumber TYSD1_HUMAN Reactome DB_ID: 9033586 UniProt:Q2T9J0 TYSND1 TYSND1 函数的酶蛋白酶介导the removal of the leader peptide from proteins containing a PTS2 target sequence and processes several PTS1-containing proteins. Catalyzes the processing of PTS1-proteins involved in the peroxisomal beta-oxidation of fatty acids.SUBUNIT Homodimer. Forms a heterodimer with the C-terminal cleavage product (45 kDa form). Forms a heterodimer with the N-terminal cleavage product (15 kDa form). Interacts with PEX5. Interacts with LONP2.PTM Self-cleavage gives rise to an N-terminal 15-kDa fragment and C-terminal 45-kDa fragment upon import into the peroxisomes. The full-lengh TYSND1 is the active the proteolytic processing of PTS1- and PTS2-proteins and in self-cleavage, and intermolecular self-cleavage of TYSND1 down-regulates its protease activity.SIMILARITY Belongs to the peptidase S1B family. UniProt Q2T9J0 1 EQUAL 566 EQUAL ReactomeDatabase ID Release 77 9033586 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033586 Reactome R-HSA-9033586 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033586.1 ReactomeDatabase ID Release 77 9033111 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033111 Reactome R-HSA-9033111 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033111.1 PEX5S,L:Cargo protein Reactome DB_ID: 9033153 1 1 ReactomeDatabase ID Release 77 9033153 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033153 Reactome R-HSA-9033153 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033153.1 ReactomeDatabase ID Release 77 9033233 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033233 Reactome R-HSA-9033233 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033233.2 22747494 Pubmed 2012 Pex5p imports folded tetrameric catalase by interaction with Pex13p Otera, Hidenori Fujiki, Yukio Traffic 13:1364-77 9837948 Pubmed 1998 The difference in recognition of terminal tripeptides as peroxisomal targeting signal 1 between yeast and human is due to different affinities of their receptor Pex5p to the cognate signal and to residues adjacent to it Lametschwandtner, G Brocard, C Fransen, M Van Veldhoven, Paul P Berger, J Hartig, A J. Biol. Chem. 273:33635-43 25854684 Pubmed 2015 The peroxisomal protein import machinery displays a preference for monomeric substrates Freitas, Marta O Francisco, Tânia Rodrigues, Tony A Lismont, Celien Domingues, Pedro Pinto, Manuel P Grou, Cláudia P Fransen, Marc Azevedo, Jorge E Open Biol 5:140236 20178365 Pubmed 2010 A proteome-wide perspective on peroxisome targeting signal 1(PTS1)-Pex5p affinities Ghosh, Debdip Berg, Jeremy M J. Am. Chem. Soc. 132:3973-9 21976670 Pubmed 2011 PEX5 protein binds monomeric catalase blocking its tetramerization and releases it upon binding the N-terminal domain of PEX14 Freitas, Marta O Francisco, Tânia Rodrigues, Tony A Alencastre, Inês S Pinto, Manuel P Grou, Cláudia P Carvalho, Andreia F Fransen, Marc Sá-Miranda, Clara Azevedo, Jorge E J. Biol. Chem. 286:40509-19 12456682 Pubmed 2003 PEX5 binds the PTS1 independently of Hsp70 and the peroxin PEX12 Harper, Courtney C Berg, Jeremy M Gould, Stephen J J. Biol. Chem. 278:7897-901 17007944 Pubmed 2006 Peroxisome targeting signal 1: is it really a simple tripeptide? Brocard, Cécile Hartig, Andreas Biochim. Biophys. Acta 1763:1565-73 9668159 Pubmed 1998 An isoform of pex5p, the human PTS1 receptor, is required for the import of PTS2 proteins into peroxisomes Braverman, N Dodt, G Gould, S J Valle, D Hum. Mol. Genet. 7:1195-205 7719337 Pubmed 1995 Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders Dodt, G Braverman, N Wong, C Moser, A Moser, H W Watkins, P Valle, D Gould, S J Nat. Genet. 9:115-25 8824437 Pubmed 1995 Characterization of the yeast and human receptors for the carboxy-terminal tripeptide peroxisomal targeting signal Wiemer, E A Terlecky, S R Nuttley, W M Subramani, S Cold Spring Harb. Symp. Quant. Biol. 60:637-48 9653144 Pubmed 1998 Identification of a human PTS1 receptor docking protein directly required for peroxisomal protein import Fransen, M Terlecky, S R Subramani, S Proc。国家的。学会科学。美国95:8087 - 92 26220973 Pubmed 2015 A novel type of rhizomelic chondrodysplasia punctata, RCDP5, is caused by loss of the PEX5 long isoform Barøy, Tuva Koster, J Strømme, Petter Ebberink, Merel S Misceo, Doriana Ferdinandusse,萨夏 Holmgren, Asbjørn Hughes, Timothy Merckoll, Else Westvik, Jostein Woldseth, Berit Walter, John Wood, Nick Tvedt, Bjørn Stadskleiv,案发ine Wanders, Ronald J A Waterham, Hans R Frengen, Eirik Hum. Mol. Genet. 24:5845-54 12559034 Pubmed 2003 Protein structure and import into the peroxisomal matrix Brocard, Cécile B Jedeszko, Christopher Song, Hong Chang Terlecky, Stanley R Walton, Paul A Traffic 4:74-82 12578380 Pubmed 2003 Correlating structure and affinity for PEX5:PTS1 complexes Gatto, Gregory J Maynard, Ernest L Guerrerio, Anthony L Geisbrecht, BV Gould, Stephen J Berg, Jeremy M Biochemistry 42:1660-6 11101887 Pubmed 2000 Peroxisomal targeting signal-1 recognition by the TPR domains of human PEX5 Gatto, G J Geisbrecht, B V Gould, S J Berg, J M Nat. Struct. Biol. 7:1091-5 22002062 Pubmed 2011 Two proteases, trypsin domain-containing 1 (Tysnd1) and peroxisomal lon protease (PsLon), cooperatively regulate fatty acid β-oxidation in peroxisomal matrix Okumoto, Kanji Kametani, Yukari Fujiki, Yukio J. Biol. Chem. 286:44367-79 7790377 Pubmed 1995 Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders Wiemer, E A Nuttley, W M Bertolaet, B L Li, X Francke, U Wheelock, M J Anné, U K Johnson, K R Subramani, S J. Cell Biol. 130:51-65 LEFT-TO-RIGHT PEX5S,L:Cargo binds PEX13:PEX14:PEX2:PEX10:PEX12 (Docking and Translocation Module) PEX5S or PEX5L bound to cargo proteins containing PTS1 interacts with the Docking and Translocation Module (PEX13:PEX14:PEX2:PEX10:PEX12) (Gould et al. 1996, Fransen et al. 1998, Will et al. 1999, Neufeld et al. 2009, Shiozawa et al. 2009, Freitas et al. 2011, Francisco et al. 2013, Neufeld et al. 2014, Dias et al. 2017). Authored: May, Bruce, 2017-12-18 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-18 DTM complex PEX13:PEX14:PEX2:PEX10:PEX12 Docking and translocation module Reactome DB_ID: 9033606 peroxisomal membrane GENE ONTOLOGY GO:0005778 PEX2:PEX10:PEX12 Peroxisomal RING finger complex ReactomeDB_ID: 8953904 PEX10 Peroxisome biogenesis factor 10 PEX10_HUMAN ReactomeDB_ID: 8953928 UniProt:O60683 PEX10 PEX10 RNF69 FUNCTION Somewhat implicated in the biogenesis of peroxisomes.SUBUNIT Interacts with PEX19.SIMILARITY Belongs to the pex2/pex10/pex12 family. UniProt O60683 1 EQUAL 326 EQUAL ReactomeDatabase ID Release 77 8953928 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953928 Reactome R-HSA-8953928 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953928.1 1 PEX12 Peroxisome assembly protein 12 PEX12_HUMAN ReactomeDB_ID: 8953911 UniProt:O00623 PEX12 PEX12 PAF3 FUNCTION Required for protein import into peroxisomes.SUBUNIT Interacts with PEX5 and PEX10. Interacts with PEX19 via its cytoplasmic domain.SIMILARITY Belongs to the pex2/pex10/pex12 family. UniProt O00623 1 EQUAL 359 EQUAL ReactomeDatabase ID Release 77 8953911 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953911 Reactome R-HSA-8953911 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953911.1 1 PEX2 Peroxisome biogenesis factor 2 PEX2_HUMAN ReactomeDB_ID: 8953913 UniProt:P28328 PEX2 PEX2 PAF1 PMP3 PMP35 PXMP3 RNF72 FUNCTION Somewhat implicated in the biogenesis of peroxisomes.SIMILARITY Belongs to the pex2/pex10/pex12 family. UniProt P28328 1 EQUAL 305 EQUAL ReactomeDatabase ID Release 77 8953913 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953913 Reactome R-HSA-8953913 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953913.1 1 ReactomeDatabase ID Release 77 8953904 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953904 Reactome R-HSA-8953904 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953904.2 1 PEX13:PEX14 Peroxisomal Docking Complex Reactome DB_ID: 9033095 PEX13 Peroxisomal membrane protein PEX13 PEX13_HUMAN ReactomeDB_ID: 452790 UniProt:Q92968 PEX13 PEX13 FUNCTION Component of the peroxisomal translocation machinery with PEX14 and PEX17. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PAS10/PEX5). Involved in the import of PTS1 and PTS2 proteins.SUBUNIT Interacts with PEX19.SIMILARITY Belongs to the peroxin-13 family.CAUTION It is uncertain whether Met-1 or Met-40 is the initiator. UniProt Q92968 1 EQUAL 403 EQUAL ReactomeDatabase ID Release 77 452790 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=452790 Reactome R-HSA-452790 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-452790.1 1 PEX14 Peroxisomal membrane protein PEX14 PEX14_HUMAN Reactome DB_ID: 9033101 UniProt:O75381 PEX14 PEX14 FUNCTION Peroxisome membrane protein that is an essential component of the peroxisomal import machinery. Functions as a docking factor for the predominantly cytoplasmic PTS1 receptor (PEX5). Plays a key role for peroxisome movement through a direct interaction with tubulin.SUBUNIT Interacts with PEX5, PEX13 and PEX19. Interacts with tubulin.SIMILARITY Belongs to the peroxin-14 family. UniProt O75381 2 EQUAL 377 EQUAL ReactomeDatabase ID Release 77 9033101 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033101 Reactome R-HSA-9033101 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033101.1 1 ReactomeDatabase ID Release 77 9033095 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033095 Reactome R-HSA-9033095 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033095.1 1 ReactomeDatabase ID Release 77 9033606 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033606 Reactome R-HSA-9033606 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033606.1 PEX5S,L:Cargo:PEX13:PEX14:PEX2:PEX10:PEX12 Reactome DB_ID: 9033114 1 1 ReactomeDatabase ID Release 77 9033114 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033114 Reactome R-HSA-9033114 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033114.1 ReactomeDatabase ID Release 77 9033236 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033236 Reactome R-HSA-9033236 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033236.2 23963456 Pubmed 2013 A cargo-centered perspective on the PEX5 receptor-mediated peroxisomal protein import pathway Francisco, Tânia Rodrigues, Tony A Freitas, Marta O Grou, Cláudia P Carvalho, Andreia F Sá-Miranda, Clara Pinto, Manuel P Azevedo, Jorge E J. Biol. Chem. 288:29151-9 10022913 Pubmed 1999 Identification and characterization of the human orthologue of yeast Pex14p Will, G K Soukupova, M Hong, X Erdmann, K S Kiel, J A Dodt, G Kunau, W H Erdmann, R Mol. Cell. Biol. 19:2265-77 19584060 Pubmed 2009 Solution structure of human Pex5.Pex14.PTS1 protein complexes obtained by small angle X-ray scattering Shiozawa, Kumiko Konarev, Petr V Neufeld, Christian Wilmanns, Matthias Svergun, Dmitri I J. Biol. Chem. 284:25334-42 24235149 Pubmed 2014 A novel Pex14 protein-interacting site of human Pex5 is critical for matrix protein import into peroxisomes Neuhaus, Alexander Kooshapur, Hamed Wolf, Janina Meyer, N Helge Madl, Tobias Saidowsky, Jürgen Hambruch, Eva Lazam, Anissa Jung, Martin Sattler, M Schliebs, Wolfgang Erdmann, Ralf J. Biol. Chem. 289:437-48 19197237 Pubmed 2009 Structural basis for competitive interactions of Pex14 with the import receptors Pex5 and Pex19 Neufeld, Christian Filipp, Fabian V 西蒙,贝恩德• Neuhaus, Alexander Schüller, Nicole David, Christine Kooshapur, Hamed Madl, Tobias Erdmann, Ralf Schliebs, Wolfgang Wilmanns, Matthias Sattler, M EMBO J. 28:745-54 28765278 Pubmed 2017 The peroxisomal matrix protein translocon is a large cavity-forming protein assembly into which PEX5 protein enters to release its cargo Dias, Ana F Rodrigues, Tony A Pedrosa, Ana G Barros-Barbosa, Aurora Francisco, Tânia Azevedo, Jorge E J. Biol. Chem. 292:15287-15300 8858165 Pubmed 1996 Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptor Gould, S J Kalish, J E Morrell, J C Bjorkman, J Urquhart, A J Crane, D I J. Cell Biol. 135:85-95 LEFT-TO-RIGHT Cargo of PEX5S,L translocates from the cytosol to the peroxisomal matrix After binding the Docking and Translocation Module comprising PEX14, PEX13, PEX2, PEX10 and PEX12 , PEX5S or PEX5L bound to a cargo protein becomes localized to the membrane (Dodt et al. 1995, Wiemer et al. 1995, Alencastre et al. 2009, Francisco et al. 2013, Dias et al. 2017). In a reaction that is not yet fully characterized, the cargo protein is released into the peroxisomal matrix while PEX5S or PEX5L remains in the membrane (Dodt et al. 1995, Wiemer et al. 1995, Alencastre et al. 2009, Franscisco et al. 2013). One model for the reaction hypothesizes that PEX13:PEX14 (associated with PEX2:PEX10:PEX12) forms a barrel in the peroxisomal membrane while PEX5S or PEX5L acts as a plunger to guide the cargo through the barrel (Dias et al. 2017, Francisco et al. 2017). Notably, the reaction does not require a source of energy such as ATP (Oliveira et al. 2003). Mutations in PEX5 cause defects in import of PTS1-containing proteins or PTS2-containing proteins or both (Eberrink et al. 2009, Barøy et al. 2015). Authored: May, Bruce, 2017-12-18 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-18 PEX5S,L:PEX13:PEX14:PEX2:PEX10:PEX12 Reactome DB_ID: 9033091 1 1 Converted from EntitySet in Reactome PEX5S,L Reactome DB_ID: 9033614 PEX5_HUMAN L PEX5对碘氧基苯甲醚 Peroxisomal targeting signal 1 receptor Reactome DB_ID: 9033489 1 EQUAL 639 EQUAL ReactomeDatabase ID Release 77 9033489 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033489 Reactome R-HSA-9033489 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033489.1 PEX5_HUMAN PEX5 isoform S Peroxisomal targeting signal 1 receptor PEX5(1-214,252-639) ReactomeDB_ID: 8953907 1 EQUAL 639 EQUAL ReactomeDatabase ID Release 77 8953907 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953907 Reactome R-HSA-8953907 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953907.2 ReactomeDatabase ID Release 77 9033614 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033614 Reactome R-HSA-9033614 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033614.1 1 ReactomeDatabase ID Release 77 9033091 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033091 Reactome R-HSA-9033091 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033091.1 Converted from EntitySet in Reactome Cargo proteins containing PTS1 Reactome DB_ID: 9033148 ACOX1 Peroxisomal acyl-coenzyme A oxidase 1 ACOX1_HUMAN ReactomeDB_ID: 2046071 peroxisomal matrix GENE ONTOLOGY GO:0005782 1 EQUAL 660 EQUAL ReactomeDatabase ID Release 77 2046071 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=2046071 Reactome R-HSA-2046071 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-2046071.1 ACOX2 Acyl-coenzyme氧化酶2,过氧化物酶病 ReactomeDB_ID: 192321 1 EQUAL 681 EQUAL ReactomeDatabase ID Release 77 192321 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=192321 Reactome R-HSA-192321 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-192321.1 ACOX3 Peroxisomal acyl-coenzyme A oxidase 3 ReactomeDB_ID: 389884 1 EQUAL 700 EQUAL ReactomeDatabase ID Release 77 389884 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=389884 Reactome R-HSA-389884 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-389884.1 EHHADH Peroxisomal bifunctional enzyme Reactome DB_ID: 400155 1 EQUAL 723 EQUAL ReactomeDatabase ID Release 77 400155 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=400155 Reactome R-HSA-400155 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-400155.1 HSD17B4 HSD17B4(1-736) Peroxisomal multifunctional enzyme type 2 hydroxysteroid (17-beta) dehydrogenase 4 DHB4_HUMAN ReactomeDB_ID: 192322 1 EQUAL 736 EQUAL ReactomeDatabase ID Release 77 192322 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=192322 Reactome R-HSA-192322 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-192322.2 SCP2 SCPx thiolase (SCPX) Non-specific lipid-transfer protein NLTP_HUMAN ReactomeDB_ID: 2046067 1 EQUAL 547 EQUAL ReactomeDatabase ID Release 77 2046067 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=2046067 Reactome R-HSA-2046067 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-2046067.1 ECI2 Enoyl-CoA delta isomerase 2, isoform 2 ReactomeDB_ID: 6809809 39 EQUAL 394 EQUAL ReactomeDatabase ID Release 77 6809809 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=6809809 Reactome R-HSA-6809809 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6809809.1 DECR2 Peroxisomal 2,4-dienoyl-CoA reductase DECR2_HUMAN ReactomeDB_ID: 6786710 2 EQUAL 292 EQUAL ReactomeDatabase ID Release 77 6786710 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=6786710 Reactome R-HSA-6786710 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6786710.1 Delta ECH1(1-328) ECH1_HUMAN Reactome DB_ID: 9033140 34 EQUAL 328 EQUAL ReactomeDatabase ID Release 77 9033140 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033140 Reactome R-HSA-9033140 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033140.1 PECR Peroxisomal trans-2-enoyl-CoA reductase ReactomeDB_ID: 6809577 2 EQUAL 303 EQUAL ReactomeDatabase ID Release 77 6809577 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=6809577 Reactome R-HSA-6809577 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6809577.1 HACL1 2-hydroxyacyl-CoA lyase 1 2-hydroxyphytanoyl-CoA lyase ReactomeDB_ID: 389600 1 EQUAL 578 EQUAL ReactomeDatabase ID Release 77 389600 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=389600 Reactome R-HSA-389600 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-389600.1 AMACR Alpha-methylacyl-CoA racemase ReactomeDB_ID: 192146 2 EQUAL 382 EQUAL ReactomeDatabase ID Release 77 192146 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=192146 Reactome R-HSA-192146 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-192146.1 CRAT carnitine acetyltransferase ReactomeDB_ID: 390293 1 EQUAL 626 EQUAL ReactomeDatabase ID Release 77 390293 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=390293 Reactome R-HSA-390293 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-390293.1 CROT Carnitine O-octanoyltransferase ReactomeDB_ID: 390296 1 EQUAL 612 EQUAL ReactomeDatabase ID Release 77 390296 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=390296 Reactome R-HSA-390296 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-390296.1 ACOT8 Acyl-coenzyme A thioesterase 8 ReactomeDB_ID: 193426 1 EQUAL 319 EQUAL ReactomeDatabase ID Release 77 193426 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=193426 Reactome R-HSA-193426 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-193426.1 ACOT2_HUMAN ACOT2(1-483) Acyl-coenzyme A thioesterase 2, mitochondrial Reactome DB_ID: 9033086 1 EQUAL 483 EQUAL ReactomeDatabase ID Release 77 9033086 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033086 Reactome R-HSA-9033086 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033086.1 CAT Catalase apoprotein (EC 1.11.1.6) CATA_HUMAN Catalase ReactomeDB_ID: 51417 2 EQUAL 527 EQUAL ReactomeDatabase ID Release 77 51417 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=51417 Reactome R-HSA-51417 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-51417.1 DAO D-amino-acid oxidase ReactomeDB_ID: 389807 1 EQUAL 347 EQUAL ReactomeDatabase ID Release 77 389807 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=389807 Reactome R-HSA-389807 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-389807.1 DDO D-aspartate oxidase ReactomeDB_ID: 6810058 1 EQUAL 341 EQUAL ReactomeDatabase ID Release 77 6810058 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=6810058 Reactome R-HSA-6810058 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6810058.1 HAO1 Hydroxyacid oxidase 1 HAOX1 ReactomeDB_ID: 389824 1 EQUAL 370 EQUAL ReactomeDatabase ID Release 77 389824 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=389824 Reactome R-HSA-389824 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-389824.1 HAO2 Hydroxyacid oxidase 2 HAOX2_HUMAN ReactomeDB_ID: 6787806 2 EQUAL 351 EQUAL ReactomeDatabase ID Release 77 6787806 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=6787806 Reactome R-HSA-6787806 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6787806.1 VLCS SLC27A2 Very long-chain acyl-CoA synthetase ReactomeDB_ID: 389579 1 EQUAL 620 EQUAL ReactomeDatabase ID Release 77 389579 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=389579 Reactome R-HSA-389579 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-389579.1 GNPAT Glycerone-phosphate O-acyltransferase Dihydroxyacetone phosphate acyltransferase DHAP-AT DAP-AT Acyl- CoA:dihydroxyacetonephosphateacyltransferase ReactomeDB_ID: 1500605 1 EQUAL 680 EQUAL ReactomeDatabase ID Release 77 1500605 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=1500605 Reactome R-HSA-1500605 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-1500605.1 IDH1 Isocitrate dehydrogenase (NADP) cytoplasmic PICD ReactomeDB_ID: 389546 1 EQUAL 414 EQUAL ReactomeDatabase ID Release 77 389546 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=389546 Reactome R-HSA-389546 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-389546.1 AGXT PXLP-AGXT Alanine-glyoxylate aminotransferase Serine-pyruvate aminotransferase ReactomeDB_ID: 389669 209 EQUAL N6-pyridoxal phosphate-L-lysine MOD MOD:00128 1 EQUAL 392 EQUAL ReactomeDatabase ID Release 77 389669 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=389669 Reactome R-HSA-389669 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-389669.1 PIPOX Peroxisomal sarcosine oxidase SOX_HUMAN ReactomeDB_ID: 452911 1 EQUAL 390 EQUAL ReactomeDatabase ID Release 77 452911 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=452911 Reactome R-HSA-452911 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-452911.1 EPHX2 Bifunctional epoxide hydrolase 2 domain recommendedName: Cytosolic epoxide hydrolase 2 HYES_HUMAN Reactome DB_ID: 9033088 1 EQUAL 555 EQUAL ReactomeDatabase ID Release 77 9033088 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033088 Reactome R-HSA-9033088 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033088.1 GSTK1 Glutathione S-transferase kappa 1 ecNumber2.5.1.18/ecNumber GSTK1_HUMAN Reactome DB_ID: 9033110 1 EQUAL 226 EQUAL ReactomeDatabase ID Release 77 9033110 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033110 Reactome R-HSA-9033110 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033110.1 PAO PAOX ReactomeDB_ID: 141345 1 EQUAL 649 EQUAL ReactomeDatabase ID Release 77 141345 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=141345 Reactome R-HSA-141345 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-141345.1 MLYCD MLYCD(40-493) malonyl-CoA decarboxylase ReactomeDB_ID: 2291936 40 EQUAL 493 EQUAL ReactomeDatabase ID Release 77 2291936 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=2291936 Reactome R-HSA-2291936 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-2291936.2 HMGCL_HUMAN HMGCL(1-325) Hydroxymethylglutaryl-CoA lyase, mitochondrial Reactome DB_ID: 9033125 1 EQUAL 325 EQUAL ReactomeDatabase ID Release 77 9033125 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033125 Reactome R-HSA-9033125 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033125.1 NUDT7 Peroxisomal coenzyme A diphosphatase NUDT7 ecNumber3.6.1.-/ecNumber NUDT7_HUMAN ReactomeDB_ID: 6809215 1 EQUAL 238 EQUAL ReactomeDatabase ID Release 77 6809215 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=6809215 Reactome R-HSA-6809215 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6809215.1 IDE Insulin-degrading enzyme ecNumber3.4.24.56/ecNumber IDE_HUMAN Reactome DB_ID: 9033157 1 EQUAL 1019 EQUAL ReactomeDatabase ID Release 77 9033157 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033157 Reactome R-HSA-9033157 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033157.1 LONP2 fullName证据= " 2 "朗蛋白酶同族体2,peroxisomal ecNumber evidence="2"3.4.21.-/ecNumber LONP2_HUMAN Reactome DB_ID: 9033144 2 EQUAL 852 EQUAL ReactomeDatabase ID Release 77 9033144 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033144 Reactome R-HSA-9033144 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033144.1 NUDT19 Nucleoside diphosphate-linked moiety X motif 19, mitochondrial NUD19_HUMAN ReactomeDB_ID: 6809219 15 EQUAL 375 EQUAL ReactomeDatabase ID Release 77 6809219 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=6809219 Reactome R-HSA-6809219 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-6809219.1 BAAT Bile acid-CoA:amino acid N-acetyltransferase ReactomeDB_ID: 193517 1 EQUAL 418 EQUAL ReactomeDatabase ID Release 77 193517 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=193517 Reactome R-HSA-193517 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-193517.1 DHRS4 Dehydrogenase/reductase SDR family member 4 ecNumber1.1.1.184/ecNumber DHRS4_HUMAN Reactome DB_ID: 9033097 1 EQUAL 278 EQUAL ReactomeDatabase ID Release 77 9033097 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033097 Reactome R-HSA-9033097 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033097.1 NOS2 Nitric oxide synthase, inducible ecNumber1.14.13.39/ecNumber NOS2_HUMAN Reactome DB_ID: 9033124 1 EQUAL 1153 EQUAL ReactomeDatabase ID Release 77 9033124 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033124 Reactome R-HSA-9033124 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033124.1 MPV17 Protein Mpv17 MPV17_HUMAN Reactome DB_ID: 9033146 1 EQUAL 176 EQUAL ReactomeDatabase ID Release 77 9033146 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033146 Reactome R-HSA-9033146 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033146.1 ACOT4 Acyl-coenzyme A thioesterase 4 ACOT4_HUMAN ReactomeDB_ID: 5690054 2 EQUAL 421 EQUAL ReactomeDatabase ID Release 77 5690054 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5690054 Reactome R-HSA-5690054 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5690054.1 TYSND1 Peroxisomal leader peptide-processing protease ecNumber3.4.21.-/ecNumber TYSD1_HUMAN ReactomeDB_ID: 8933309 1 EQUAL 566 EQUAL ReactomeDatabase ID Release 77 8933309 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8933309 Reactome R-HSA-8933309 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8933309.1 ReactomeDatabase ID Release 77 9033148 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033148 Reactome R-HSA-9033148 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033148.2 ReactomeDatabase ID Release 77 9033235 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033235 Reactome R-HSA-9033235 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033235.2 18712838 Pubmed 2009 Genotype-phenotype correlation in PEX5-deficient peroxisome biogenesis defective cell lines Ebberink, Merel S Mooyer, Petra A W Koster, J Dekker, Conny J M Eyskens, François J M Dionisi-Vici, Carlo Clayton, Peter T Barth, Peter G Wanders, Ronald J A Waterham, Hans R Hum. Mutat. 30:93-8 19632994 Pubmed 2009 Mapping the cargo protein membrane translocation step into the PEX5 cycling pathway Alencastre, Inês S Rodrigues, Tony A Grou, Cláudia P Fransen, Marc Sá-Miranda, Clara Azevedo, Jorge E J. Biol. Chem. 284:27243-51 12885776 Pubmed 2003 The energetics of Pex5p-mediated peroxisomal protein import Oliveira, Marcia E Gouveia, Alexandra M Pinto, Rui A Sá-Miranda, Clara Azevedo, Jorge E J. Biol. Chem. 278:39483-8 28787099 Pubmed 2017 Protein transport into peroxisomes: Knowns and unknowns Francisco, Tânia Rodrigues, Tony A Dias, Ana F Barros-Barbosa, Aurora Bicho, Diana Azevedo, Jorge E Bioessays 39:10.1002/bies.201700047 LEFT-TO-RIGHT PEX2:PEX10:PEX12 binds PEX5S,L (in PEX5S:PEX13:PEX14) and Ub:UBE2D1,2,3 A RING E3 ubiquitin ligase complex containing PEX10, PEX12, and PEX2 ubiquitinates PEX5L. The PEX2:PEX10:PEX12 complex is believed to bind an activated E2-ubiquitin conjugate (one of Ub:UBE2D1, Ub:UBE2D2, Ub:UBE2D3) and PEX5L in a complex that also contains PEX13 and PEX14 (Chang et al. 1999, Carvalho et al. 2007, Grou et al. 2008, Grou et al. 2009, Okumoto et al. 2011). The short isoform of PEX5, PEX5S, is inferred to undergo the same reaction. Authored: May, Bruce, 2017-01-01 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-01-01 Ub:UBE2D1,2,3 ReactomeDB_ID: 8953915 Converted from EntitySet in Reactome Ub-C85-UBE2D1,2,3 ReactomeDB_ID: 8963780 UbcH5/Ubc4 Ub-C85-UBE2D1 ReactomeDB_ID: 8864160 UniProt:P51668 UBE2D1 UBE2D1 SFT UBC5A UBCH5 UBCH5A FUNCTION Accepts ubiquitin from the E1 complex and catalyzes its covalent attachment to other proteins (PubMed:22496338). In vitro catalyzes 'Lys-48'-linked polyubiquitination (PubMed:20061386). Mediates the selective degradation of short-lived and abnormal proteins. Functions in the E6/E6-AP-induced ubiquitination of p53/TP53. Mediates ubiquitination of PEX5 and auto-ubiquitination of STUB1, TRAF6 and TRIM63/MURF1 (PubMed:18042044, PubMed:18359941). Ubiquitinates STUB1-associated HSP90AB1 in vitro (PubMed:18042044). Lacks inherent specificity for any particular lysine residue of ubiquitin (PubMed:18042044). Essential for viral activation of IRF3 (PubMed:19854139). Mediates polyubiquitination of CYP3A4 (PubMed:19103148).PATHWAY Protein modification; protein ubiquitination.SUBUNIT Component of a E3 ubiquitin ligase complex containing UBE2D1, SIAH1, CACYBP/SIP, SKP1, APC and TBL1X. Interacts with RNF11.TISSUE SPECIFICITY Ubiquitous. Up-regulated in livers of iron-overloaded patients with hereditary hemochromatosis.PTM Autoubiquitinated in vitro.SIMILARITY Belongs to the ubiquitin-conjugating enzyme family.CAUTION PubMed:9362508 cloned and sequenced SFT which consisted of UBE2D1 last coding exon along with intronic sequences on the 5'-end of this exon. A function in iron transport has been described. UniProt P51668 85 EQUAL S-(glycyl)-L-cysteine (Cys-Gly) MOD MOD:00211 1 EQUAL 147 EQUAL ReactomeDatabase ID Release 77 8864160 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8864160 Reactome R-HSA-8864160 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8864160.1 UbcH5b Ub-C85-UBE2D2 Ubiquitin-conjugating enzyme E2 D2 UB2D2_HUMAN ReactomeDB_ID: 8864214 UniProt:P62837 UBE2D2 UBE2D2 PUBC1 UBC4 UBC5B UBCH4 UBCH5B FUNCTION Accepts ubiquitin from the E1 complex and catalyzes its covalent attachment to other proteins. In vitro catalyzes 'Lys-48'-linked polyubiquitination. Mediates the selective degradation of short-lived and abnormal proteins. Functions in the E6/E6-AP-induced ubiquitination of p53/TP53. Mediates ubiquitination of PEX5 and autoubiquitination of STUB1 and TRAF6. Involved in the signal-induced conjugation and subsequent degradation of NFKBIA, FBXW2-mediated GCM1 ubiquitination and degradation, MDM2-dependent degradation of p53/TP53 and the activation of MAVS in the mitochondria by DDX58/RIG-I in response to viral infection. Essential for viral activation of IRF3.PATHWAY Protein modification; protein ubiquitination.SUBUNIT Interacts with SCF (SKP1-CUL1-F-box protein) E3 ubiquitin ligase complex. Interacts with CNOT4 (via RING domain). Interacts with E3 ubiquitin-protein ligases CBLC, PJA1 and PJA2. Interacts with PDZRN3. Interacts with PPP1R11 (By similarity).SIMILARITY Belongs to the ubiquitin-conjugating enzyme family. UniProt P62837 85 EQUAL 1 EQUAL 147 EQUAL ReactomeDatabase ID Release 77 8864214 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8864214 Reactome R-HSA-8864214 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8864214.1 UbcH5c Ub-C85-UBE2D3 Ubiquitin-conjugating enzyme E2 D3 UB2D3_HUMAN ReactomeDB_ID: 8864191 UniProt:P61077 UBE2D3 UBE2D3 UBC5C UBCH5C FUNCTION Accepts ubiquitin from the E1 complex and catalyzes its covalent attachment to other proteins. In vitro catalyzes 'Lys-11'-, as well as 'Lys-48'-linked polyubiquitination. Cooperates with the E2 CDC34 and the SCF(FBXW11) E3 ligase complex for the polyubiquitination of NFKBIA leading to its subsequent proteasomal degradation. Acts as an initiator E2, priming the phosphorylated NFKBIA target at positions 'Lys-21' and/or 'Lys-22' with a monoubiquitin. Ubiquitin chain elongation is then performed by CDC34, building ubiquitin chains from the UBE2D3-primed NFKBIA-linked ubiquitin. Acts also as an initiator E2, in conjunction with RNF8, for the priming of PCNA. Monoubiquitination of PCNA, and its subsequent polyubiquitination, are essential events in the operation of the DNA damage tolerance (DDT) pathway that is activated after DNA damage caused by UV or chemical agents during S-phase. Associates with the BRCA1/BARD1 E3 ligase complex to perform ubiquitination at DNA damage sites following ionizing radiation leading to DNA repair. Targets DAPK3 for ubiquitination which influences promyelocytic leukemia protein nuclear body (PML-NB) formation in the nucleus. In conjunction with the MDM2 and TOPORS E3 ligases, functions ubiquitination of p53/TP53. Supports NRDP1-mediated ubiquitination and degradation of ERBB3 and of BRUCE which triggers apoptosis. In conjunction with the CBL E3 ligase, targets EGFR for polyubiquitination at the plasma membrane as well as during its internalization and transport on endosomes. In conjunction with the STUB1 E3 quality control E3 ligase, ubiquitinates unfolded proteins to catalyze their immediate destruction. Together with RNF135, catalyzes the viral RNA-dependent 'Lys-63'-linked polyubiquitination of RIG-I/DDX58 to activate the downstream signaling pathway that leads to interferon beta production (PubMed:28469175).PATHWAY Protein modification; protein ubiquitination.SUBUNIT Interacts with SCF (SKP1-CUL1-F-box protein) E3 ubiquitin ligase complex; when Cullin is neddylated, the interaction between the E2 and the SCF complex is strengthened. Interacts with DAPK3. Interacts with BRCA1; the DNA damage checkpoint promotes the association with BRCA1 after ionizing radiation. Interacts non-covalently with ubiquitin. Interacts with E3 ubiquitin-protein ligase CBLC. Interacts with UBTD1 (PubMed:24211586). Interacts with DDX58 and RNF135; involved in DDX58 ubiquitination and activation (PubMed:28469175).PTM Phosphorylated by AURKB.SIMILARITY Belongs to the ubiquitin-conjugating enzyme family. UniProt P61077 85 EQUAL 1 EQUAL 147 EQUAL ReactomeDatabase ID Release 77 8864191 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8864191 Reactome R-HSA-8864191 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8864191.1 ReactomeDatabase ID Release 77 8963780 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8963780 Reactome R-HSA-8963780 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8963780.1 1 Converted from EntitySet in Reactome Ub ubiquitin ReactomeDB_ID: 113595 RPS27A RPS27A(1-76) ubiquitin (RPS27A) ReactomeDB_ID: 939205 UniProt:P62979 RPS27A RPS27A UBA80 UBCEP1 SUBUNIT Ribosomal protein S27a is part of the 40S ribosomal subunit.MISCELLANEOUS Ubiquitin is encoded by 4 different genes. UBA52 and RPS27A genes code for a single copy of ubiquitin fused to the ribosomal proteins L40 and S27a, respectively. UBB and UBC genes code for a polyubiquitin precursor with exact head to tail repeats, the number of repeats differ between species and strains.MISCELLANEOUS For a better understanding, features related to ubiquitin are only indicated for the first chain.SIMILARITY In the N-terminal section; belongs to the ubiquitin family.SIMILARITY In the C-terminal section; belongs to the eukaryotic ribosomal protein eS31 family. UniProt P62979 1 EQUAL 76 EQUAL ReactomeDatabase ID Release 77 939205 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939205 Reactome R-HSA-939205 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939205.1 UBA52 UBA52(1-76) ubiquitin (UBA52) ReactomeDB_ID: 939203 UniProt:P62987 UBA52 UBA52 UBCEP2 SUBUNIT Ribosomal protein L40 is part of the 60S ribosomal subunit. Interacts with UBQLN1 (via UBA domain).MISCELLANEOUS Ubiquitin is encoded by 4 different genes. UBA52 and RPS27A genes code for a single copy of ubiquitin fused to the ribosomal proteins L40 and S27a, respectively. UBB and UBC genes code for a polyubiquitin precursor with exact head to tail repeats, the number of repeats differ between species and strains.MISCELLANEOUS For a better understanding, features related to ubiquitin are only indicated for the first chain.SIMILARITY In the N-terminal section; belongs to the ubiquitin family.SIMILARITY In the C-terminal section; belongs to the eukaryotic ribosomal protein eL40 family. UniProt P62987 1 EQUAL 76 EQUAL ReactomeDatabase ID Release 77 939203 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939203 Reactome R-HSA-939203 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939203.1 UBB UBB(1-76) 泛素(UBB 1) ReactomeDB_ID: 939214 UniProt:P0CG47 UBB UBB SUBUNIT Interacts with SKP1-KMD2A and SKP1-KMD2B complexes.MISCELLANEOUS Ubiquitin is encoded by 4 different genes. UBA52 and RPS27A genes code for a single copy of ubiquitin fused to the ribosomal proteins L40 and S27a, respectively. UBB and UBC genes code for a polyubiquitin precursor with exact head to tail repeats, the number of repeats differ between species and strains.MISCELLANEOUS The mRNA encoding variant UBB(+1) is produced by an unknown mechanism involving the deletion of a GT dinucleotide in the close proximity of a GAGAG motif (PubMed:9422699). This variant mRNA is found in normal brain, but the encoded protein accumulates only in brain neurofibrillary tangles and neuritic plaques in Alzheimer disease and other tauopathies, as well as polyglutaminopathies (PubMed:14597671). UBB(+1) variant cannot be used for polyubiquitination, is not effectively degraded by the proteasome when ubiquitinated and ubiquitinated UBB(+1) is refractory to disassembly by deubiquitinating enzymes (DUBs). In healthy brain, UBB(+1) C-terminus can be cleaved by UCHL3 (PubMed:21762696).MISCELLANEOUS For a better understanding, features related to ubiquitin are only indicated for the first chain.SIMILARITY Belongs to the ubiquitin family. UniProt P0CG47 1 EQUAL 76 EQUAL ReactomeDatabase ID Release 77 939214 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939214 Reactome R-HSA-939214 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939214.1 UBB(77-152) ubiquitin (UBB 2) ReactomeDB_ID: 939213 77 EQUAL 152 EQUAL ReactomeDatabase ID Release 77 939213 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939213 Reactome R-HSA-939213 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939213.1 UBB(153-228) ubiquitin (UBB 3) ReactomeDB_ID: 939230 153 EQUAL 228 EQUAL ReactomeDatabase ID Release 77 939230 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939230 Reactome R-HSA-939230 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939230.1 UBC UBC(1-76) ubiquitin (UBC 1) ReactomeDB_ID: 939188 UniProt:P0CG48 UBC UBC MISCELLANEOUS Ubiquitin is encoded by 4 different genes. UBA52 and RPS27A genes code for a single copy of ubiquitin fused to the ribosomal proteins L40 and S27a, respectively. UBB and UBC genes code for a polyubiquitin precursor with exact head to tail repeats, the number of repeats differ between species and strains.MISCELLANEOUS For the sake of clarity sequence features are annotated only for the first chain, and are not repeated for each of the following chains.SIMILARITY Belongs to the ubiquitin family. UniProt P0CG48 1 EQUAL 76 EQUAL ReactomeDatabase ID Release 77 939188 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939188 Reactome R-HSA-939188 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939188.1 UBC(77-152) ubiquitin (UBC 2) ReactomeDB_ID: 939164 77 EQUAL 152 EQUAL ReactomeDatabase ID Release 77 939164 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939164 Reactome R-HSA-939164 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939164.1 UBC(153-228) ubiquitin (UBC 3) ReactomeDB_ID: 939258 153 EQUAL 228 EQUAL ReactomeDatabase ID Release 77 939258 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939258 Reactome R-HSA-939258 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939258.1 UBC(229-304) ubiquitin (UBC 4) ReactomeDB_ID: 939192 229 EQUAL 304 EQUAL ReactomeDatabase ID Release 77 939192 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939192 Reactome R-HSA-939192 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939192.1 UBC(305-380) ubiquitin (UBC 5) ReactomeDB_ID: 939232 305 EQUAL 380 EQUAL ReactomeDatabase ID Release 77 939232 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939232 Reactome R-HSA-939232 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939232.1 UBC(381-456) ubiquitin (UBC 6) ReactomeDB_ID: 939191 381 EQUAL 456 EQUAL ReactomeDatabase ID Release 77 939191 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939191 Reactome R-HSA-939191 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939191.1 UBC(457-532) ubiquitin (UBC 7) ReactomeDB_ID: 939184 457 EQUAL 532 EQUAL ReactomeDatabase ID Release 77 939184 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939184 Reactome R-HSA-939184 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939184.1 UBC(533-608) 泛素(哥伦比亚大学8) ReactomeDB_ID: 939239 533 EQUAL 608 EQUAL ReactomeDatabase ID Release 77 939239 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939239 Reactome R-HSA-939239 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939239.1 UBC(609-684) ubiquitin (UBC 9) ReactomeDB_ID: 939165 609 EQUAL 684 EQUAL ReactomeDatabase ID Release 77 939165 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=939165 Reactome R-HSA-939165 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-939165.1 ReactomeDatabase ID Release 77 113595 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=113595 Reactome R-HSA-113595 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-113595.1 1 ReactomeDatabase ID Release 77 8953915 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953915 Reactome R-HSA-8953915 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953915.1 PEX2:PEX10:PEX12:PEX5S,L:Ub:UBE2D1,2,3:PEX13:PEX14 ReactomeDB_ID: 8953942 1 1 ReactomeDatabase ID Release 77 8953942 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953942 Reactome R-HSA-8953942 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953942.1 ReactomeDatabase ID Release 77 8953917 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953917 Reactome R-HSA-8953917 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953917.2 21554508 Pubmed 2011 Cysteine ubiquitination of PTS1 receptor Pex5p regulates Pex5p recycling Okumoto, Kanji Misono, Sachi Miyata, Non Matsumoto, Yui Mukai, Satoru Fujiki, Yukio Traffic 12:1067-83 10562279 Pubmed 1999 PEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein import Chang, C C Warren, D S Sacksteder, K A Gould, S J J. Cell Biol. 147:761-74 18359941 Pubmed 2008 Members of the E2D (UbcH5) family mediate the ubiquitination of the conserved cysteine of Pex5p, the peroxisomal import receptor Grou, Cláudia P Carvalho, Andreia F Pinto, Manuel P Wiese, Sebastian Piechura, Heike Meyer, Helmut E Warscheid, Bettina Sá-Miranda, Clara Azevedo, Jorge E J. Biol. Chem. 283:14190-7 19208625 Pubmed 2009 Properties of the ubiquitin-pex5p thiol ester conjugate Grou, Cláudia P Carvalho, Andreia F Pinto, Manuel P Huybrechts, Sofie J Sá-Miranda, Clara Fransen, M Azevedo, Jorge E J. Biol. Chem. 284:10504-13 17726030 Pubmed 2007 Ubiquitination of mammalian Pex5p, the peroxisomal import receptor Carvalho, Andreia F Pinto, Manuel P Grou, Cláudia P Alencastre, Inês S Fransen, Marc Sá-Miranda, Clara Azevedo, Jorge E J. Biol. Chem. 282:31267-72 LEFT-TO-RIGHT PEX2:PEX10:PEX12 monoubiquitinates PEX5S,L at cysteine-11 的环形E3泛素连接酶sub-complex PEX2:PEX10:PEX12 catalyzes the transfer of ubiquitin from an E2-ubiquitin conjugate (one of Ub:UBE2D1, Ub:UBE2D2, or Ub:UBE2D3) to the cysteine-11 residue of the substrate PEX5L, the peroxisomal matrix protein shuttling receptor (Carvalho et al. 2007; Grou et al. 2008, Okumoto et al. 2011, Sargent et al. 2016, inferred from yeast in Dodt and Gould 1996). The thiol ester bond between ubiquitin and the cysteine residue of PEX5 is unusual among ubiquitin substrates, which usually have isopeptide bonds between ubiquitin and a lysine residue. Monoubiquitination of PEX5 at cysteine-11 is an integral and mandatory step in the PEX5-mediated peroxisomal protein transport pathway; in its absence, PEX5 cannot be extracted from the peroxisomal membrane docking/translocation machinery (the peroxisomal protein translocon), and thus transport of newly synthesized peroxisomal matrix proteins to the organelle matrix stops (Grou et al. 2009). In addition to monoubiquitinating PEX5 during peroxisomal protein import, the PEX2:PEX10:PEX12 complex has also been implicated in pexophagy, a type of selective autophagy targeting peroxisomes. Pexophagy seems to be triggered mainly by ubiquitination of PEX5, which, in this case, can occur either at its cysteine-11 or lysine-209 residues, but ubiquitination of ABCD3 (also known as PMP70) and other peroxisomal membrane proteins may also be involved (Zhang et al. 2015, inferred from mouse in Nordgren et al. 2015, Sargent et al. 2016). Authored: May, Bruce, 2017-01-01 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-01-01 PEX2:PEX10:PEX12:Ub:PEX5S,L:PEX13:PEX14 Reactome DB_ID: 9033528 1 Converted from EntitySet in Reactome Ub:PEX5S,L Reactome DB_ID: 9033604 Ub:PEX5L Reactome DB_ID: 9033498 1 PEX5_HUMAN Ub-C11-PEX5L Peroxisomal targeting signal 1 receptor Reactome DB_ID: 9033501 11 EQUAL 1 EQUAL 639 EQUAL ReactomeDatabase ID Release 77 9033501 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033501 Reactome R-HSA-9033501 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033501.1 1 ReactomeDatabase ID Release 77 9033498 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033498 Reactome R-HSA-9033498 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033498.1 Ub:PEX5S ReactomeDB_ID: 8953945 PEX5_HUMAN Ub-C11-PEX5S Peroxisomal targeting signal 1 receptor ReactomeDB_ID: 8953944 11 EQUAL 1 EQUAL 639 EQUAL ReactomeDatabase ID Release 77 8953944 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953944 Reactome R-HSA-8953944 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953944.2 1 1 ReactomeDatabase ID Release 77 8953945 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953945 Reactome R-HSA-8953945 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953945.2 ReactomeDatabase ID Release 77 9033604 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033604 Reactome R-HSA-9033604 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033604.1 1 1 ReactomeDatabase ID Release 77 9033528 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033528 Reactome R-HSA-9033528 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033528.1 Converted from EntitySet in Reactome UBE2D1,2,3 UbcH5a,b,c ReactomeDB_ID: 1234120 UBE2D1 UbcH5/Ubc4 Reactome DB_ID: 174236 1 EQUAL 147 EQUAL ReactomeDatabase ID Release 77 174236 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=174236 Reactome R-HSA-174236 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-174236.1 UBE2D2 UbcH5b Ubiquitin-conjugating enzyme E2 D2 UB2D2_HUMAN ReactomeDB_ID: 934628 1 EQUAL 147 EQUAL ReactomeDatabase ID Release 77 934628 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=934628 Reactome R-HSA-934628 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-934628.1 UBE2D3 UbcH5c Ubiquitin-conjugating enzyme E2 D3 UB2D3_HUMAN ReactomeDB_ID: 934630 1 EQUAL 147 EQUAL ReactomeDatabase ID Release 77 934630 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=934630 Reactome R-HSA-934630 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-934630.1 ReactomeDatabase ID Release 77 1234120 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=1234120 Reactome R-HSA-1234120 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-1234120.1 ACTIVATION GENE ONTOLOGY GO:0061630 gene ontology term for cellular function MI MI:0355 Same Catalyst Activity ReactomeDatabase ID Release 77 8953956 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953956 ReactomeDatabase ID Release 77 8953946 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=8953946 Reactome R-HSA-8953946 4 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-8953946.4 8991089 Pubmed 1996 Multiple PEX genes are required for proper subcellular distribution and stability of Pex5p, the PTS1 receptor: evidence that PTS1 protein import is mediated by a cycling receptor Dodt, G Gould, S J J. Cell Biol. 135:1763-74 26344566 Pubmed 2015 ATM functions at the peroxisome to induce pexophagy in response to ROS Zhang, Jiangwei Tripathi, Durga Nand Jing, Ji Alexander, Angela Kim, Jinhee Powell, Reid T Dere, Ruhee Tait-Mulder, Jacqueline Lee, Ji-Hoon Paull, Tanya T Pandita, Raj K Charaka, Vijaya K Pandita, Tej K Kastan, Michael B Walker, Cheryl Lyn Nat. Cell Biol. 17:1259-69 26086376 Pubmed 2015 Export-deficient monoubiquitinated PEX5 triggers peroxisome removal in SV40 large T antigen-transformed mouse embryonic fibroblasts Nordgren, Marcus Francisco, Tânia Lismont, Celien Hennebel, Lore Brees, Chantal Wang, Bo Van Veldhoven, Paul P Azevedo, Jorge E Fransen, Marc Autophagy 11:1326-40 27597759 Pubmed 2016 PEX2 is the E3 ubiquitin ligase required for pexophagy during starvation Sargent, Graeme van Zutphen, Tim Shatseva, Tatiana Zhang, Ling Di Giovanni, Valeria Bandsma, Robert Kim, Peter Kijun J. Cell Biol. 214:677-90 24662292 Pubmed 2014 Distinct modes of ubiquitination of peroxisome-targeting signal type 1 (PTS1) receptor Pex5p regulate PTS1 protein import Okumoto, Kanji Noda, Hiromi Fujiki, Yukio J. Biol. Chem. 289:14089-108 LEFT-TO-RIGHT PEX2:PEX10:PEX12:Ub:PEX5S,L:PEX13:PEX14 binds PEX1:PEX6:PEX26 and ZFAND6 PEX1:PEX6:PEX26 (also known as the Receptor Export Module or peroxisomal AAA ATPase complex) extracts ubiquitinated PEX5S or PEX5L in the peroxisomal membrane (Tamura et al. 2006, Tamura et al. 2014). PEX1 and PEX6 are soluble proteins that form a hexameric ring bound to PEX26 in the peroxisomal membrane (Matsumoto et al. 2003, Weller et al. 2005). ZFAND6 (AWP1) probably binds to ubiquitinated PEX5 and PEX6 and acts as an export factor (Miyata et al. 2012). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 PEX1:PEX6:PEX26 Reactome DB_ID: 9033100 PEX26 Peroxisome assembly protein 26 PEX26_HUMAN Reactome DB_ID: 9033092 UniProt:Q7Z412 PEX26 PEX26 FUNCTION Probably required for protein import into peroxisomes. Anchors PEX1 and PEX6 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes. Involved in the import of catalase and proteins containing a PTS2 target sequence, but not in import of proteins with a PTS1 target sequence.SUBUNIT Interacts directly with PEX6 via its cytoplasmic domain. Interacts indirectly with PEX1, via its interaction with PEX6.TISSUE SPECIFICITY Widely expressed. Highly expressed in kidney, liver, brain and skeletal muscles. Expressed at intermediate level in pancreas, placenta and heart. Weakly expressed in lung.SIMILARITY Belongs to the peroxin-26 family. UniProt Q7Z412 1 EQUAL 305 EQUAL ReactomeDatabase ID Release 77 9033092 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033092 Reactome R-HSA-9033092 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033092.1 1 PEX1 Peroxisome biogenesis factor 1 PEX1_HUMAN Reactome DB_ID: 9033116 UniProt:O43933 PEX1 PEX1 FUNCTION Required for stability of PEX5 and protein import into the peroxisome matrix. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes.SUBUNIT Interacts directly with PEX6. Interacts indirectly with PEX26, via its interaction with PEX6.SIMILARITY Belongs to the AAA ATPase family. UniProt O43933 1 EQUAL 1283 EQUAL ReactomeDatabase ID Release 77 9033116 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033116 Reactome R-HSA-9033116 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033116.1 3 PEX6 Peroxisome assembly factor 2 PEX6_HUMAN Reactome DB_ID: 9033104 UniProt:Q13608 PEX6 PEX6 PXAAA1 FUNCTION Involved in peroxisome biosynthesis. Required for stability of the PTS1 receptor. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes.SUBUNIT Interacts directly with PEX26 and PEX1. Mediates the indirect interaction between PEX1 and PEX26. Interacts with ZFAND6.TISSUE SPECIFICITY Expressed in the retina, at higher levels in the photoreceptor layer at the joint between the outer and inner segments.SIMILARITY Belongs to the AAA ATPase family. UniProt Q13608 1 EQUAL 980 EQUAL ReactomeDatabase ID Release 77 9033104 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033104 Reactome R-HSA-9033104 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033104.1 3 ReactomeDatabase ID Release 77 9033100 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033100 Reactome R-HSA-9033100 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033100.1 ZFAND6 AN1-type zinc finger protein 6 ZFAN6_HUMAN Reactome DB_ID: 9033138 UniProt:Q6FIF0 ZFAND6 ZFAND6 AWP1 ZA20D3 HT032 FUNCTION Involved in regulation of TNF-alpha induced NF-kappa-B activation and apoptosis. Involved in modulation of 'Lys-48'-linked polyubiquitination status of TRAF2 and decreases association of TRAF2 with RIPK1. Required for PTS1 target sequence-dependent protein import into peroxisomes and PEX5 stability; may cooperate with PEX6. In vitro involved in PEX5 export from the cytosol to peroxisomes (By similarity).SUBUNIT Interacts with PKN1 (By similarity). Interacts with TRAF2. Interacts with mono- and polyubiquitin. Interacts with PEX6. Interacts with PEX5 (Cys-linked ubiquitinated) (By similarity).TISSUE SPECIFICITY Widely expressed with high level in heart, skeletal muscle, liver, kidney and placenta.DOMAIN The A20-type zinc finger domain mediates regulation of NF-kappa-B activity.DOMAIN The AN1-type zinc finger domain mediates association with TRAF2. UniProt Q6FIF0 1 EQUAL 208 EQUAL ReactomeDatabase ID Release 77 9033138 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033138 Reactome R-HSA-9033138 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033138.1 PEX2:PEX10:PEX12:Ub:PEX5S,L:PEX1:PEX6:PEX26:ZFAND6 Reactome DB_ID: 9033488 1 1 1 ReactomeDatabase ID Release 77 9033488 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033488 Reactome R-HSA-9033488 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033488.1 ReactomeDatabase ID Release 77 9033533 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033533 Reactome R-HSA-9033533 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033533.2 12717447 Pubmed 2003 The pathogenic peroxin Pex26p recruits the Pex1p-Pex6p AAA ATPase complexes to peroxisomes Matsumoto, Naomi Tamura, Shigehiko Fujiki, Yukio Nat. Cell Biol. 5:454-60 21980954 Pubmed 2012 AWP1/ZFAND6 functions in Pex5 export by interacting with cys-monoubiquitinated Pex5 and Pex6 AAA ATPase Miyata, Non Okumoto, Kanji Mukai, Satoru Noguchi, Masafumi Fujiki, Yukio Traffic 13:168-83 25016021 Pubmed 2014 AAA peroxins and their recruiter Pex26p modulate the interactions of peroxins involved in peroxisomal protein import Tamura, Shigehiko Matsumoto, Naomi Takeba, Ryota Fujiki, Yukio J. Biol. Chem. 289:24336-46 15858711 Pubmed 2005 Alternative splicing suggests extended function of PEX26 in peroxisome biogenesis Weller, Sabine Cajigas, Ivelisse Morrell, James Obie, Cassandra Steel, Gary Gould, Stephen J Valle, David Am. J. Hum. Genet. 76:987-1007 16854980 Pubmed 2006 Dynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26p Tamura, Shigehiko Yasutake, Shinobu Matsumoto, Naomi Fujiki, Yukio J. Biol. Chem. 281:27693-704 LEFT-TO-RIGHT 7.2.2 PEX1:PEX6:PEX26:ZFAND6:Ub:PEX5S,L:PEX14:PEX13:PEX2:PEX10:PEX12 dissociates yielding cytosolic Ub:PEX5S,L and membrane PEX14:PEX13:PEX2:PEX10:PEX12 Ubiquitinated PEX5 isoform S or isoform L (Ub:PEX5S,L) is released from the peroxisomal membrane and interaction with the Docking and Translocation Module by PEX1:PEX6:PEX26:ZFAND6 (the peroxisomal AAA ATPase complex, receptor export module) (Tamura et al. 2014, Law et al. 2017, also inferred from yeast homologs). PEX1 and PEX6 form a cytosolic hexameric ring that is anchored to the peroxisomal membrane by PEX26. Hydrolysis of ATP by PEX1 and PEX6 appears to cause a conformational change in PEX1:PEX6:PEX26:ZFAND6 that releases Ub:PEX5S,L from the peroxisomal membrane and into the cytosol (reviewed in Saffert et al. 2017). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 ATP Adenosine 5'-triphosphate ATP(4-) ReactomeDB_ID: 113592 ATP(4-) [ChEBI:30616] ATP(4-) ATP atp Adenosine 5'-triphosphate ChEBI CHEBI:30616 ReactomeDatabase ID Release 77 113592 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=113592 Reactome R-ALL-113592 5 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-113592.5 COMPOUND C00002 additional information MI MI:0361 ADP Adenosine 5'-diphosphate ADP(3-) ReactomeDB_ID: 29370 ADP(3-) [ChEBI:456216] ADP(3-) ADP 5'-O-[(phosphonatooxy)phosphinato]adenosine ADP trianion ChEBI CHEBI:456216 ReactomeDatabase ID Release 77 29370 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=29370 Reactome R-ALL-29370 5 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29370.5 COMPOUND C00008 Converted from EntitySet in Reactome Ub:PEX5S,L Reactome DB_ID: 9033619 Ub:PEX5L Reactome DB_ID: 9033504 PEX5_HUMAN Ub-C11-PEX5L Peroxisomal targeting signal 1 receptor Reactome DB_ID: 9033510 1 EQUAL 639 EQUAL ReactomeDatabase ID Release 77 9033510 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033510 Reactome R-HSA-9033510 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033510.1 1 1 ReactomeDatabase ID Release 77 9033504 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033504 Reactome R-HSA-9033504 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033504.1 Ub:PEX5S Reactome DB_ID: 9033492 1 PEX5_HUMAN Ub-C11-PEX5S Peroxisomal targeting signal 1 receptor Reactome DB_ID: 9033479 1 EQUAL 639 EQUAL ReactomeDatabase ID Release 77 9033479 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033479 Reactome R-HSA-9033479 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033479.1 1 ReactomeDatabase ID Release 77 9033492 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033492 Reactome R-HSA-9033492 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033492.1 ReactomeDatabase ID Release 77 9033619 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033619 Reactome R-HSA-9033619 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033619.1 ACTIVATION GENE ONTOLOGY GO:0042626 ReactomeDatabase ID Release 77 9033905 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033905 ReactomeDatabase ID Release 77 9033505 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033505 Reactome R-HSA-9033505 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033505.3 9539740 Pubmed 1998 Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I Tamura, S Okumoto, K Toyama, R Shimozawa, N Tsukamoto, T Suzuki, Y Osumi, T Kondo, N Fujiki, Y Proc。国家的。学会科学。U.S.A. 95:4350-5 28521612 Pubmed 2017 The peroxisomal AAA ATPase complex prevents pexophagy and development of peroxisome biogenesis disorders Law, Kelsey B Bronte-Tinkew, Dana Di Pietro, Erminia Snowden, Ann Jones, Richard O Moser, Ann Brumell, John H Braverman, N Kim, Peter K Autophagy 13:868-884 28611990 Pubmed 2017 Structure and Function of p97 and Pex1/6 Type II AAA+ Complexes Saffert, Paul Enenkel, Cordula Wendler, Petra Front Mol Biosci 4:33 LEFT-TO-RIGHT USP9X binds Ub:PEX5S The deubiquitinating enzyme USP9X binds cytosolic ubiquitinated PEX5S (Ub:PEX5S) and then hydrolyzes the thioester bond between the carboxyl terminus of ubiquitin and cysteine-11 of PEX5S (inferred from the large isoform of PEX5 in Grou et al. 2012). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 FAM USP9X Probable ubiquitin carboxyl-terminal hydrolase FAF-X ReactomeDB_ID: 870499 UniProt:Q93008 USP9X USP9X DFFRX FAM USP9 函数Deubiquitinase涉及两个过程ssing of ubiquitin precursors and of ubiquitinated proteins. May therefore play an important regulatory role at the level of protein turnover by preventing degradation of proteins through the removal of conjugated ubiquitin. Specifically hydrolyzes 'Lys-48'-, 'Lys-29'- and 'Lys-33'-linked polyubiquitins chains. Essential component of TGF-beta/BMP signaling cascade. Specifically deubiquitinates monoubiquitinated SMAD4, opposing the activity of E3 ubiquitin-protein ligase TRIM33. Deubiquitinates alkylation repair enzyme ALKBH3. OTUD4 recruits USP7 and USP9X to stabilize ALKBH3, thereby promoting the repair of alkylated DNA lesions (PubMed:25944111). Regulates chromosome alignment and segregation in mitosis by regulating the localization of BIRC5/survivin to mitotic centromeres. Involved in axonal growth and neuronal cell migration (PubMed:16322459, PubMed:18254724, PubMed:19135894, PubMed:24607389). Regulates cellular clock function by enhancing the protein stability and transcriptional activity of the core circadian protein ARNTL/BMAL1 via its deubiquitinating activity (PubMed:29626158).SUBUNIT Interacts with SMAD4, MARK4, NUAK1 and BIRC5/survivin. Interacts with DCX. Interacts with OTUD4 and USP7; the interaction is direct (PubMed:25944111). Interacts with ARNTL/BMAL1 (PubMed:29626158).TISSUE SPECIFICITY Widely expressed in embryonic and adult tissues.MISCELLANEOUS Escapes X-inactivation.SIMILARITY Belongs to the peptidase C19 family. UniProt Q93008 1 EQUAL 2570 EQUAL ReactomeDatabase ID Release 77 870499 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=870499 Reactome R-HSA-870499 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-870499.1 USP9X:Ub:PEX5S Reactome DB_ID: 9033494 1 1 ReactomeDatabase ID Release 77 9033494 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033494 Reactome R-HSA-9033494 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033494.1 ReactomeDatabase ID Release 77 9033526 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033526 Reactome R-HSA-9033526 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033526.2 22371489 Pubmed 2012 Identification of ubiquitin-specific protease 9X (USP9X) as a deubiquitinase acting on ubiquitin-peroxin 5 (PEX5) thioester conjugate Grou, Cláudia P Francisco, Tânia Rodrigues, Tony A Freitas, Marta O Pinto, Manuel P Carvalho, Andreia F Domingues, Pedro Wood, Stephen A Rodríguez-Borges, José E Sá-Miranda, Clara Fransen, Marc Azevedo, Jorge E J. Biol. Chem. 287:12815-27 LEFT-TO-RIGHT 3.4.19.12 USP9X hydrolyzes Ub:PEX5S yielding PEX5S and Ubiquitin The deubiquitinating enzyme USP9X hydrolyzes the thioester bond between the carboxyl terminus of ubiquitin and cysteine-11 of PEX5S (inferred from the large isoform of PEX5L in Grou et al. 2012). The thioester bond is unstable and appears also to be spontaneously disrupted by nucleophilic attack of small metabolites such as reduced glutathione (Grou et al. 2009). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 H2O water ReactomeDB_ID: 29356 water [ChEBI:15377] water ChEBI CHEBI:15377 ReactomeDatabase ID Release 77 29356 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=29356 Reactome R-ALL-29356 5 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-29356.5 COMPOUND C00001 ACTIVATION activeUnit: #Protein127 GENE ONTOLOGY GO:0004843 ReactomeDatabase ID Release 77 9033480 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033480 ReactomeDatabase ID Release 77 9033478 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033478 Reactome R-HSA-9033478 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033478.3 LEFT-TO-RIGHT PEX7 binds cargo proteins containing PTS2 胞质PEX7绑定过氧物酶体目标信号2(PTS2), a sequence of nine amino acid residues functionally identified in 3 human peroxisomal matrix proteins (Braverman et al. 1997, Motley et al. 1997, Purdue et al. 1997, Braverman et al. 2000, Ghys et al. 2002, Motley et al. 2002, Kunze et al. 2011, Kunze et al. 2015). According to molecular modeling, the PTS2 consensus sequence binds a groove in PEX7 (Kunze et al. 2011). Mutations in PEX7 cause rhizomelic chondrodysplasia punctata type 1 (Braverman et al. 1997, Motley et al. 1997, Purdue et al. 1997). Authored: May, Bruce, 2017-12-15 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-15 PEX7 Peroxisomal targeting signal 2 receptor PEX7_HUMAN Reactome DB_ID: 9033107 UniProt:O00628 PEX7 PEX7 PTS2R FUNCTION Binds to the N-terminal PTS2-type peroxisomal targeting signal and plays an essential role in peroxisomal protein import.SUBUNIT Interacts with PEX5 (By similarity). Interacts with VWA8 (PubMed:30204880).TISSUE SPECIFICITY Ubiquitous. Highest expression in pancreas, skeletal muscle and heart.SIMILARITY Belongs to the WD repeat peroxin-7 family. UniProt O00628 1 EQUAL 323 EQUAL ReactomeDatabase ID Release 77 9033107 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033107 Reactome R-HSA-9033107 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033107.1 Converted from EntitySet in Reactome Cargo proteins containing PTS2 Reactome DB_ID: 9033139 THIK_HUMAN ACAA1(1-424) 3-ketoacyl-CoA thiolase, peroxisomal ecNumber2.3.1.16/ecNumber Reactome DB_ID: 9033127 UniProt:P09110 ACAA1 ACAA1 ACAA PTHIO FUNCTION Responsible for the thiolytic cleavage of straight chain 3-oxoacyl-CoAs. Catalyzes the cleavage of short, medium and long straight chain 3-oxoacyl-CoAs, medium chain 3-oxoacyl-CoAs being the best substrates.PATHWAY Lipid metabolism; fatty acid metabolism.SUBUNIT Homodimer.INDUCTION Peroxisomal thiolase is markedly induced (at the level of transcription) by various hypolipidemic compounds in parallel with the other two enzymes of the peroxisomal beta-oxidation system.SIMILARITY Belongs to the thiolase-like superfamily. Thiolase family. UniProt P09110 1 EQUAL 424 EQUAL ReactomeDatabase ID Release 77 9033127 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033127 Reactome R-HSA-9033127 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033127.1 ADAS_HUMAN AGPS(1-658) Alkyldihydroxyacetonephosphate合成酶,每oxisomal Reactome DB_ID: 9033119 UniProt:O00116 AGPS AGPS AAG5 催化酰基链的交换功能in acyl-dihydroxyacetonephosphate (acyl-DHAP) for a long chain fatty alcohol, yielding the first ether linked intermediate, i.e. alkyl-dihydroxyacetonephosphate (alkyl-DHAP), in the pathway of ether lipid biosynthesis.PATHWAY Glycerolipid metabolism; ether lipid biosynthesis.SUBUNIT Homodimer.SIMILARITY Belongs to the FAD-binding oxidoreductase/transferase type 4 family. UniProt O00116 59 EQUAL 658 EQUAL ReactomeDatabase ID Release 77 9033119 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033119 Reactome R-HSA-9033119 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033119.1 PAHX_HUMAN PHYH(1-338) Phytanoyl-CoA dioxygenase, peroxisomal ecNumber1.14.11.18/ecNumber Reactome DB_ID: 9033143 UniProt:O14832 PHYH PHYH PAHX FUNCTION Catalyzes the 2-hydroxylation of not only racemic phytanoyl-CoA and the isomers of 3-methylhexadecanoyl-CoA, but also a variety of other mono-branched 3-methylacyl-CoA esters (with a chain length of at least seven carbon atoms) and straight-chain acyl-CoA esters (with a chain length longer than four carbon atoms) (PubMed:9326939, PubMed:10744784, PubMed:12031666, PubMed:12923223). Does not hydroxylate long and very long straight chain acyl-CoAs or 2-methyl- and 4-methyl-branched acyl-CoAs (PubMed:10744784, PubMed:12923223).PATHWAY Lipid metabolism; fatty acid metabolism.SUBUNIT Interacts with FKBP52 (PubMed:10051602). Interacts with PHYHIP.TISSUE SPECIFICITY Expressed in liver, kidney, and T-cells, but not in spleen, brain, heart, lung and skeletal muscle.SIMILARITY Belongs to the PhyH family. UniProt O14832 1 EQUAL 338 EQUAL ReactomeDatabase ID Release 77 9033143 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033143 Reactome R-HSA-9033143 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033143.1 ReactomeDatabase ID Release 77 9033139 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033139 Reactome R-HSA-9033139 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033139.1 PEX7:Cargo proteins Reactome DB_ID: 9033142 1 1 ReactomeDatabase ID Release 77 9033142 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033142 Reactome R-HSA-9033142 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033142.1 ReactomeDatabase ID Release 77 9033232 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033232 Reactome R-HSA-9033232 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033232.2 11931631 Pubmed 2002 Functional studies on human Pex7p: subcellular localization and interaction with proteins containing a peroxisome-targeting signal type 2 and other peroxins Ghys, Karen Fransen, Marc Mannaerts, Guy P Van Veldhoven, Paul P Biochem. J. 365:41-50 9090381 Pubmed 1997 Human PEX7 encodes the peroxisomal PTS2 receptor and is responsible for rhizomelic chondrodysplasia punctata Braverman, N Steel, G Obie, C Moser, A Moser, H Gould, S J Valle, D Nat. Genet. 15:369-76 9090383 Pubmed 1997 Rhizomelic chondrodysplasia punctata is caused by deficiency of human PEX7, a homologue of the yeast PTS2 receptor Purdue, P E Zhang, J W Skoneczny, M Lazarow, P B Nat. Genet. 15:381-4 25538232 Pubmed 2015 Mechanistic insights into PTS2-mediated peroxisomal protein import: the co-receptor PEX5L drastically increases the interaction strength between the cargo protein and the receptor PEX7 Kunze, Markus Malkani, Naila Maurer-Stroh, Sebastian Wiesinger, Christoph Schmid, Johannes A Berger, Johannes J. Biol. Chem. 290:4928-40 10673331 Pubmed 2000 PEX7 gene structure, alternative transcripts, and evidence for a founder haplotype for the frequent RCDP allele, L292ter Braverman, N Steel, G Lin, P Moser, A Moser, H Valle, D Genomics 63:181-92 11781871 Pubmed 2002 Mutational spectrum in the PEX7 gene and functional analysis of mutant alleles in 78 patients with rhizomelic chondrodysplasia punctata type 1 Motley, Alison M Brites, Pedro Gerez, Lisya Hogenhout, Eveline Haasjes, Janet Benne, R Tabak, Henk F Wanders, Ronald J A Waterham, Hans R Am. J. Hum. Genet. 70:612-24 22057399 Pubmed 2011 Structural requirements for interaction of peroxisomal targeting signal 2 and its receptor PEX7 Kunze, Markus Neuberger, Georg Maurer-Stroh, Sebastian Ma, Jianmin Eck, Thomas Braverman, N Schmid, Johannes A Eisenhaber, Frank Berger, Johannes J. Biol. Chem. 286:45048-62 9090382 Pubmed 1997 Rhizomelic chondrodysplasia punctata is a peroxisomal protein targeting disease caused by a non-functional PTS2 receptor Motley, A M Hettema, E H Hogenhout, E M Brites, P ten Asbroek, A L Wijburg, F A Baas, F Heijmans, H S Tabak, H F Wanders, R J Distel, B Nat. Genet. 15:377-80 LEFT-TO-RIGHT PEX5L binds PEX7:Cargo protein The long isoform of PEX5, PEX5L, binds PEX7 that is already bound to a PTS2-containing cargo protein (Braverman et al. 1998, Dodt et al. 2001, Kunze et al. 2015, Rodrigues et al. 2015). The binding of PEX5L to PEX7 increases the affinity of PEX7 for cargo protein (Kunze et al. 2015). Mutations affecting the additional sequence present only in the long isoform of PEX5 cause rhizomelic chondrodysplasia punctata type 5 (Baroy et al. 2015). Authored: May, Bruce, 2017-12-15 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-15 PEX5L:PEX7:Cargo protein Reactome DB_ID: 9033115 1 1 ReactomeDatabase ID Release 77 9033115 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033115 Reactome R-HSA-9033115 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033115.1 ReactomeDatabase ID Release 77 9033240 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033240 Reactome R-HSA-9033240 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033240.2 26138649 Pubmed 2015 回顾的traperoxisomal pathway of mammalian PEX7 Rodrigues, Tony A Grou, Cláudia P Azevedo, Jorge E Sci Rep 5:11806 11546814 Pubmed 2001 Domain mapping of human PEX5 reveals functional and structural similarities to Saccharomyces cerevisiae Pex18p and Pex21p Dodt, G Warren, D Becker, E Rehling, P Gould, S J J. Biol. Chem. 276:41769-81 LEFT-TO-RIGHT PEX5L:PEX7:Cargo binds PEX13:PEX14:PEX2:PEX10:PEX12 (Docking and Translocation Module) PEX5L bound to PEX7:Cargo interacts with the peroxisomal membrane complex PEX13:PEX14:PEX2:PEX10:PEX12 (the Docking-Translocation Complex) thus bringing PEX7 and its cargo to dock at the peroxisomal membrane (Gould et al. 1996, Fransen et al. 1998, Will et al. 1999, Dodt et al. 2001, Rodrigues et al. 2014, Rodrigues et al. 2015, also inferred from hamster and rat homologues). Authored: May, Bruce, 2017-12-18 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-18 PEX5L:PEX7:Cargo:PEX13:PEX14:PEX2:PEX10:PEX12 Reactome DB_ID: 9033117 1 1 ReactomeDatabase ID Release 77 9033117 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033117 Reactome R-HSA-9033117 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033117.1 ReactomeDatabase ID Release 77 9033238 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033238 Reactome R-HSA-9033238 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033238.2 24865970 Pubmed 2014 A PEX7-centered perspective on the peroxisomal targeting signal type 2-mediated protein import pathway Rodrigues, Tony A Alencastre, Inês S Francisco, Tânia Brites, Pedro Fransen, Marc Grou, Cláudia P Azevedo, Jorge E Mol. Cell. Biol. 34:2917-28 LEFT-TO-RIGHT Cargo of PEX5L:PEX7 translocates from the cytosol to the peroxisomal matrix 货物蛋白质绑定到PEX7释放PEX7 into the peroxisomal matrix in a reaction that does not require ATP (Purdue et al. 1997, Dodt et al. 2001, Rodrigues et al. 2014, Rodrigues et al. 2015). PEX7 may also be released into the matrix (inferred from yeast in Nair et al. 2004), however later research indicates that PEX7 remains with PEX5L in the peroxisomal membrane (Rodrigues et al. 2015) apparently in a proteinaceous cavity (Dias et al. 2017). Mutations in PEX5 cause defects in import of PTS1-containing proteins or PTS2-containing proteins or both (Eberrink et al. 2009, Barøy et al. 2015). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 Converted from EntitySet in Reactome Cargo proteins containing PTS2 Reactome DB_ID: 9033482 THIK_HUMAN ACAA1(1-424) 3-ketoacyl-CoA thiolase, peroxisomal ecNumber2.3.1.16/ecNumber Reactome DB_ID: 9033517 1 EQUAL 424 EQUAL ReactomeDatabase ID Release 77 9033517 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033517 Reactome R-HSA-9033517 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033517.1 ADAS_HUMAN AGPS(1-658) Alkyldihydroxyacetonephosphate合成酶,每oxisomal Reactome DB_ID: 9033523 1 EQUAL 658 EQUAL ReactomeDatabase ID Release 77 9033523 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033523 Reactome R-HSA-9033523 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033523.1 PAHX_HUMAN PHYH(1-338) Phytanoyl-CoA dioxygenase, peroxisomal ecNumber1.14.11.18/ecNumber Reactome DB_ID: 9033535 1 EQUAL 338 EQUAL ReactomeDatabase ID Release 77 9033535 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033535 Reactome R-HSA-9033535 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033535.1 ReactomeDatabase ID Release 77 9033482 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033482 Reactome R-HSA-9033482 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033482.1 PEX5L:PEX7:PEX13:PEX14:PEX2:PEX10:PEX12 Reactome DB_ID: 9033513 1 1 PEX7 Peroxisomal targeting signal 2 receptor PEX7_HUMAN Reactome DB_ID: 9033493 1 EQUAL 323 EQUAL ReactomeDatabase ID Release 77 9033493 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033493 Reactome R-HSA-9033493 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033493.1 1 1 ReactomeDatabase ID Release 77 9033513 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033513 Reactome R-HSA-9033513 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033513.1 ReactomeDatabase ID Release 77 9033514 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033514 Reactome R-HSA-9033514 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033514.2 15545321 Pubmed 2004 Pex7p translocates in and out of peroxisomes in Saccharomyces cerevisiae Nair, Devi M Purdue, P Edward Lazarow, Paul B J. Cell Biol. 167:599-604 LEFT-TO-RIGHT PEX2:PEX10:PEX12 binds PEX5L (in PEX5L:PEX7:PEX13:PEX14:PEX2:PEX10:PEX12) and Ub:UBE2D1,2,3 A RING E3 ubiquitin ligase sub-complex containing PEX10, PEX12, and PEX2 ubiquitinates PEX5L. PEX10:PEX12:PEX2 is believed to bind an activated E2-ubiquitin conjugate (one of Ub:UBE2D1, Ub:UBE2D2, Ub:UBE2D3) and PEX5L in a complex that also contains PEX13 and PEX14 (Chang et al. 1999, Carvalho et al. 2007, Grou et al. 2008, Grou et al. 2009, Okumoto et al. 2011). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 PEX2:PEX10:PEX12:PEX5L:Ub:UBE2D1,2,3:PEX7:PEX13:PEX14 Reactome DB_ID: 9033521 1 1 ReactomeDatabase ID Release 77 9033521 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033521 Reactome R-HSA-9033521 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033521.1 ReactomeDatabase ID Release 77 9033527 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033527 Reactome R-HSA-9033527 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033527.2 LEFT-TO-RIGHT PEX2:PEX10:PEX12 monoubiquitinates PEX5L at cysteine-11 的环形E3泛素连接酶sub-complex PEX2:PEX10:PEX12 catalyzes the transfer of ubiquitin from an E2-ubiquitin conjugate (one of Ub:UBE2D1, Ub:UBE2D2, or Ub:UBE2D3) to the cysteine-11 residue of the substrate PEX5L, the peroxisomal matrix protein shuttling receptor (Carvalho et al. 2007; Grou et al. 2008, Okumoto et al. 2011, Sargent et al. 2016, inferred from yeast in Dodt and Gould 1996). In contrast to PEX5, PEX7 transiently associated with the docking and translocation module (which comprises PEX14, PEX13, PEX2, PEX10, and PEX12) is not ubiquitinated. The thiol ester bond between ubiquitin and the cysteine residue of PEX5 is unusual among ubiquitin substrates, which usually have isopeptide bonds between ubiquitin and a lysine residue. Monoubiquitination of PEX5 at cysteine-11 is an integral and mandatory step in the PEX5-mediated peroxisomal protein transport pathway; in its absence, PEX5 and PEX7 cannot be extracted from the peroxisomal membrane docking-translocation machinery (the peroxisomal protein translocon), and thus transport of newly synthesized peroxisomal matrix proteins to the organelle matrix stops (Grou et al. 2009). In addition to monoubiquitinating PEX5 during peroxisomal protein import, the PEX2:PEX10:PEX12 sub-complex has also been implicated in pexophagy, a type of selective autophagy targeting peroxisomes. Pexophagy seems to be triggered mainly by ubiquitination of PEX5, which, in this case, can occur either at its cysteine-11 or lysine-209 residues, but ubiquitination of ABCD3 (also known as PMP70) and other peroxisomal membrane proteins may also be involved (Zhang et al. 2015, inferred from mouse in Nordgren et al. 2015, Sargent et al. 2016). Authored: May, Bruce, 2017-01-01 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-01-01 PEX2:PEX10:PEX12:Ub:PEX5L:PEX7:PEX13:PEX14 Reactome DB_ID: 9033497 1 1 1 1 ReactomeDatabase ID Release 77 9033497 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033497 Reactome R-HSA-9033497 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033497.1 ACTIVATION ReactomeDatabase ID Release 77 9034385 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9034385 ReactomeDatabase ID Release 77 9033485 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033485 Reactome R-HSA-9033485 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033485.3 LEFT-TO-RIGHT PEX2:PEX10:PEX12:Ub:PEX5L:PEX7:PEX13:PEX14 binds PEX1:PEX6:PEX26 and ZFAND6 PEX1:PEX6:PEX26 (known as the Receptor Export Module) extracts ubiquitinated PEX5L from the peroxisomal membrane Docking and Translocation Module (Tamura et al. 2006, Tamura et al. 2014). PEX1 and PEX6 are soluble proteins that form a hexameric ring bound to PEX26 in the peroxisomal membrane (Matsumoto et al. 2003, Welle et al. 2005). ZFAND6 (AWP1) probably binds to ubiquitinated PEX5 and PEX6 and acts as an export factor (Miyata et al. 2012). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 PEX2:PEX10:PEX12:Ub:PEX5L:PEX7:PEX13:PEX14:PEX1:PEX6:PEX26:ZFAND6 Reactome DB_ID: 9033532 1 1 1 ReactomeDatabase ID Release 77 9033532 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033532 Reactome R-HSA-9033532 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033532.1 ReactomeDatabase ID Release 77 9033516 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033516 Reactome R-HSA-9033516 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033516.2 LEFT-TO-RIGHT 7.2.2 PEX1:PEX6:PEX26:ZFAND6 dissociates Ub:PEX5L and PEX7 from PEX14:PEX13:PEX2:PEX10:PEX12 and translocates PEX5L and PEX7 from the peroxisomal membrane to the cytosol Ubiquitinated PEX5 isoform L (Ub:PEX5L) is released from the peroxisomal membrane Docking and Translocation Module by PEX1:PEX6:PEX26 (the peroxisomal AAA ATPase complex, receptor export module) (Tamura et al. 2014, Law et al. 2017, also inferred from yeast homologs). PEX1 and PEX6 form a cytosolic hexameric ring that is anchored to the peroxisomal membrane by PEX26. Hydrolysis of ATP by PEX1 and PEX6 appears to cause a conformational change in PEX1:PEX6:PEX26 that removes Ub:PEX5L from the peroxisomal membrane and into the cytosol (reviewed in Saffert et al. 2017). ZFAND6 probably binds ubiquitinated PEX5 and PEX6 and acts as an export factor (Miyata et al. 2012). Export of PEX7 back to the cytosol requires export of PEX5L but PEX7 and PEX5L appear to be exported separately (Rodrigues et al. 2014). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 ACTIVATION ReactomeDatabase ID Release 77 9033893 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033893 ReactomeDatabase ID Release 77 9033499 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033499 Reactome R-HSA-9033499 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033499.3 LEFT-TO-RIGHT USP9X binds Ub:PEX5L The deubiquitinating enzyme USP9X binds ubiquitinated PEX5L (ubiquitin conjugated to the large isoform of PEX5, Ub:PEX5L) and then hydrolyzes the thioester bond between the carboxyl terminus of ubiquitin and cysteine-11 of PEX5L (Grou et al. 2012). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 USP9X:Ub:PEX5L Reactome DB_ID: 9033512 1 1 ReactomeDatabase ID Release 77 9033512 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033512 Reactome R-HSA-9033512 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033512.1 ReactomeDatabase ID Release 77 9033509 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033509 Reactome R-HSA-9033509 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033509.2 LEFT-TO-RIGHT 3.4.19.12 USP9X hydrolyzes Ub:PEX5L yielding PEX5L and Ubiquitin The deubiquitinating enzyme USP9X hydrolyzes the thioester bond between the carboxyl terminus of ubiquitin and cysteine-11 of PEX5L (Grou et al. 2012). The thioester bond is unstable and appears to be also spontaneously (non-enzymatically) disrupted by nucleophilic attack of small metabolites such as reduced glutathione (Grou et al. 2009). Authored: May, Bruce, 2017-12-19 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-19 ACTIVATION activeUnit: #Protein127 ReactomeDatabase ID Release 77 9033483 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033483 ReactomeDatabase ID Release 77 9033491 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033491 Reactome R-HSA-9033491 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033491.3 TYSND1 cleaves peroxisomal proteins After proteins are imported into the peroxisome a subset of proteins are cleaved by the protease TYSND1 (Okumoto et al. 2011). Based onmutagenesis of human TYSND1 (Okumoto et al. 2011) and the homolog in Arabidopsis (Schuhmann et al. 2008), TYSND1 appears to be a trypsin-like serine protease containing a conserved histidine aspartate serine triad essential for catalysis. Mice lacking Tysnd1 have reduced peroxisomal localization of some peroxisomal enzymes and exhibit reduced beta-oxidation of fatty acids and metabolism of phytanic acid (Mizuno et al. 2013). Male mice lacking Tysnd1 are sterile due to sperm that lack acrosomal caps. Authored: May, Bruce, 2017-12-22 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-22 LEFT-TO-RIGHT 3.4.21.1 3.4.21.92 3.4.21.73 3.4.21.71 3.4.21.93 3.4.21.94 3.4.21.34 3.4.21.78 3.4.21.9 3.4.21.53 3.4.21.6 3.4.21.75 3.4.24.3 3.4.21.10 3.4.21.54 3.4.21.7 3.4.21.4 3.4.21.59 3.4.21.38 3.4.21.5 3.4.21.35 3.4.21.79 3.4.21.36 3.4.19.1 3.4.21.62 3.4.21.41 3.4.21.61 3.4.21.83 3.4.21.22 3.4.21.88 3.4.21.45 3.4.21.89 3.4.21.20 3.4.21.42 3.4.21.21 3.4.21.43 3.4.21.87 3.4.21.26 3.4.21.48 3.4.24.34 3.4.21.27 3.4.21.46 3.4.21.68 3.4.21.47 3.4.21.69 3.4.21.39 3.4.24.7 3.4.21.102 TYSND1 cleaves ACAA1 The trypsin-like serine-type endopeptidase TYSND1 cleaves ACAA1 between amino acid residues 26 and 27, yielding ACAA1(1-26) and ACAA1(27-424) (Okumoto et al. 2011). In mice, knockout of Tysnd1 causes reduced localization of Acaa1 to the peroxisome. Authored: May, Bruce, 2017-12-22 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-22 H2O water ReactomeDB_ID: 141343 ReactomeDatabase ID Release 77 141343 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=141343 Reactome R-ALL-141343 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-141343.3 ACAA1 Acetyl-Coenzyme A acyltransferase 1 3-ketoacyl-CoA thiolase, peroxisomal ReactomeDB_ID: 390238 27 EQUAL 424 EQUAL ReactomeDatabase ID Release 77 390238 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=390238 Reactome R-HSA-390238 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-390238.1 THIK_HUMAN ACAA1(1-26) 3-ketoacyl-CoA thiolase, peroxisomal ecNumber2.3.1.16/ecNumber Reactome DB_ID: 9033534 1 EQUAL 26 EQUAL ReactomeDatabase ID Release 77 9033534 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033534 Reactome R-HSA-9033534 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033534.1 ACTIVATION GENE ONTOLOGY GO:0004252 ReactomeDatabase ID Release 77 9033507 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033507 ReactomeDatabase ID Release 77 9033529 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033529 Reactome R-HSA-9033529 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033529.3 LEFT-TO-RIGHT 3.4.21.1 3.4.21.92 3.4.21.73 3.4.21.71 3.4.21.93 3.4.21.94 3.4.21.34 3.4.21.78 3.4.21.9 3.4.21.53 3.4.21.6 3.4.21.75 3.4.24.3 3.4.21.10 3.4.21.54 3.4.21.7 3.4.21.4 3.4.21.59 3.4.21.38 3.4.21.5 3.4.21.35 3.4.21.79 3.4.21.36 3.4.19.1 3.4.21.62 3.4.21.41 3.4.21.61 3.4.21.83 3.4.21.22 3.4.21.88 3.4.21.45 3.4.21.89 3.4.21.20 3.4.21.42 3.4.21.21 3.4.21.43 3.4.21.87 3.4.21.26 3.4.21.48 3.4.24.34 3.4.21.27 3.4.21.46 3.4.21.68 3.4.21.47 3.4.21.69 3.4.21.39 3.4.24.7 3.4.21.102 TYSND1 cleaves ACOX1 The trypsin-like serine-type endopeptidase TYSND1 cleaves ACOX1 between amino acid residues 438 and 439, yielding ACOX1(1-438) and ACOX1(439-660) (Okumoto et al. 2011, also inferred from mouse and rat homologs). Authored: May, Bruce, 2017-12-22 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-22 ACOX1_HUMAN ACOX1(1-438) Peroxisomal acyl-coenzyme A oxidase 1 Reactome DB_ID: 9033531 1 EQUAL 438 EQUAL ReactomeDatabase ID Release 77 9033531 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033531 Reactome R-HSA-9033531 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033531.1 ACOX1_HUMAN ACOX1(439-660) Peroxisomal acyl-coenzyme A oxidase 1 Reactome DB_ID: 9033502 439 EQUAL 660 EQUAL ReactomeDatabase ID Release 77 9033502 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033502 Reactome R-HSA-9033502 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033502.1 ACTIVATION ReactomeDatabase ID Release 77 9033515 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033515 Reactome R-HSA-9033515 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033515.3 LEFT-TO-RIGHT 3.4.21.1 3.4.21.92 3.4.21.73 3.4.21.71 3.4.21.93 3.4.21.94 3.4.21.34 3.4.21.78 3.4.21.9 3.4.21.53 3.4.21.6 3.4.21.75 3.4.24.3 3.4.21.10 3.4.21.54 3.4.21.7 3.4.21.4 3.4.21.59 3.4.21.38 3.4.21.5 3.4.21.35 3.4.21.79 3.4.21.36 3.4.19.1 3.4.21.62 3.4.21.41 3.4.21.61 3.4.21.83 3.4.21.22 3.4.21.88 3.4.21.45 3.4.21.89 3.4.21.20 3.4.21.42 3.4.21.21 3.4.21.43 3.4.21.87 3.4.21.26 3.4.21.48 3.4.24.34 3.4.21.27 3.4.21.46 3.4.21.68 3.4.21.47 3.4.21.69 3.4.21.39 3.4.24.7 3.4.21.102 TYSND1 cleaves AGPS The trypsin-like serine-type endopeptidase TYSND1 cleaves AGPS between amino acid residues 58 and 59, yielding AGPS(1-58) and AGPS(59-658) (inferred from mouse homologs). In mice, knockout of TYSND1 causes reduced localization of AGPS to the peroxisome. Authored: May, Bruce, 2017-12-22 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-22 AGPS(1-58) Alkyldihydroxyacetonephosphate合成酶,每oxisomal ADAS_HUMAN Reactome DB_ID: 9033519 1 EQUAL 58 EQUAL ReactomeDatabase ID Release 77 9033519 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033519 Reactome R-HSA-9033519 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033519.1 AGPS Alkylglycerone phosphate synthase Alkyldihydroxyacetonephosphate synthase precursor Alkyl-DHAP synthase Alkyldihydroxyacetonephosphate合成酶,每oxisomal precursor ReactomeDB_ID: 49699 59 EQUAL 658 EQUAL ReactomeDatabase ID Release 77 49699 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=49699 Reactome R-HSA-49699 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-49699.1 ACTIVATION ReactomeDatabase ID Release 77 9033506 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033506 Reactome R-HSA-9033506 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033506.3 LEFT-TO-RIGHT 3.4.21.1 3.4.21.92 3.4.21.73 3.4.21.71 3.4.21.93 3.4.21.94 3.4.21.34 3.4.21.78 3.4.21.9 3.4.21.53 3.4.21.6 3.4.21.75 3.4.24.3 3.4.21.10 3.4.21.54 3.4.21.7 3.4.21.4 3.4.21.59 3.4.21.38 3.4.21.5 3.4.21.35 3.4.21.79 3.4.21.36 3.4.19.1 3.4.21.62 3.4.21.41 3.4.21.61 3.4.21.83 3.4.21.22 3.4.21.88 3.4.21.45 3.4.21.89 3.4.21.20 3.4.21.42 3.4.21.21 3.4.21.43 3.4.21.87 3.4.21.26 3.4.21.48 3.4.24.34 3.4.21.27 3.4.21.46 3.4.21.68 3.4.21.47 3.4.21.69 3.4.21.39 3.4.24.7 3.4.21.102 TYSND1 cleaves SCP2 The trypsin-like serine-type endopeptidase TYSND1 cleaves SCP2 (also known as SCPX) between amino acid residues 424 and 425, yielding SCP2(1-424) and SCP2(425-547) (Okumoto et al. 2011, also inferred from mouse homologs). Authored: May, Bruce, 2017-12-22 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-22 NLTP_HUMAN SCP2(1-424) Non-specific lipid-transfer protein Reactome DB_ID: 9033486 1 EQUAL 424 EQUAL ReactomeDatabase ID Release 77 9033486 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033486 Reactome R-HSA-9033486 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033486.1 NLTP_HUMAN SCP2(425-547) Non-specific lipid-transfer protein Reactome DB_ID: 9033522 425 EQUAL 547 EQUAL ReactomeDatabase ID Release 77 9033522 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033522 Reactome R-HSA-9033522 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033522.1 ACTIVATION ReactomeDatabase ID Release 77 9033524 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033524 Reactome R-HSA-9033524 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033524.3 LEFT-TO-RIGHT 3.4.21.1 3.4.21.92 3.4.21.73 3.4.21.71 3.4.21.93 3.4.21.94 3.4.21.34 3.4.21.78 3.4.21.9 3.4.21.53 3.4.21.6 3.4.21.75 3.4.24.3 3.4.21.10 3.4.21.54 3.4.21.7 3.4.21.4 3.4.21.59 3.4.21.38 3.4.21.5 3.4.21.35 3.4.21.79 3.4.21.36 3.4.19.1 3.4.21.62 3.4.21.41 3.4.21.61 3.4.21.83 3.4.21.22 3.4.21.88 3.4.21.45 3.4.21.89 3.4.21.20 3.4.21.42 3.4.21.21 3.4.21.43 3.4.21.87 3.4.21.26 3.4.21.48 3.4.24.34 3.4.21.27 3.4.21.46 3.4.21.68 3.4.21.47 3.4.21.69 3.4.21.39 3.4.24.7 3.4.21.102 TYSND1 cleaves PHYH The trypsin-like serine-type endopeptidase TYSND1 cleaves PHYH between amino acid residues 30 and 31, yielding PHYH(1-30) and PHYH(31-338). In mice, knockout of Tysnd1 causes reduced localization of Phyh to peroxisomes. Authored: May, Bruce, 2017-12-22 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-22 PHYH Phytanoyl-CoA dioxygenase PAHX ReactomeDB_ID: 389574 31 EQUAL 338 EQUAL ReactomeDatabase ID Release 77 389574 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=389574 Reactome R-HSA-389574 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-389574.1 PHYH(1-30) Phytanoyl-CoA dioxygenase, peroxisomal ecNumber1.14.11.18/ecNumber PAHX_HUMAN Reactome DB_ID: 9033518 1 EQUAL 30 EQUAL ReactomeDatabase ID Release 77 9033518 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033518 Reactome R-HSA-9033518 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033518.1 ACTIVATION ReactomeDatabase ID Release 77 9033490 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033490 Reactome R-HSA-9033490 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033490.3 LEFT-TO-RIGHT 3.4.21.1 3.4.21.92 3.4.21.73 3.4.21.71 3.4.21.93 3.4.21.94 3.4.21.34 3.4.21.78 3.4.21.9 3.4.21.53 3.4.21.6 3.4.21.75 3.4.24.3 3.4.21.10 3.4.21.54 3.4.21.7 3.4.21.4 3.4.21.59 3.4.21.38 3.4.21.5 3.4.21.35 3.4.21.79 3.4.21.36 3.4.19.1 3.4.21.62 3.4.21.41 3.4.21.61 3.4.21.83 3.4.21.22 3.4.21.88 3.4.21.45 3.4.21.89 3.4.21.20 3.4.21.42 3.4.21.21 3.4.21.43 3.4.21.87 3.4.21.26 3.4.21.48 3.4.24.34 3.4.21.27 3.4.21.46 3.4.21.68 3.4.21.47 3.4.21.69 3.4.21.39 3.4.24.7 3.4.21.102 TYSND1 cleaves TYSND1 The trypsin-like serine-type endopeptidase TYSND1 cleaves TYSND1 between amino acid residues 110 and 111, yielding TYSND1(1-110) and TYSND1(111-566) (Okumoto et al. 2011). Self-cleavage of TYSND1 reduces its proteolytic activity. Authored: May, Bruce, 2017-12-22 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-22 TYSD1_HUMAN TYSND1(1-110) Peroxisomal leader peptide-processing protease ecNumber3.4.21.-/ecNumber Reactome DB_ID: 9033587 1 EQUAL 110 EQUAL ReactomeDatabase ID Release 77 9033587 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033587 Reactome R-HSA-9033587 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033587.1 TYSD1_HUMAN TYSND1(111-566) Peroxisomal leader peptide-processing protease ecNumber3.4.21.-/ecNumber Reactome DB_ID: 9033589 111 EQUAL 566 EQUAL ReactomeDatabase ID Release 77 9033589 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033589 Reactome R-HSA-9033589 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033589.1 ACTIVATION ReactomeDatabase ID Release 77 9033520 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033520 Reactome R-HSA-9033520 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033520.3 LEFT-TO-RIGHT 3.4.21.1 3.4.21.92 3.4.21.73 3.4.21.71 3.4.21.93 3.4.21.94 3.4.21.34 3.4.21.78 3.4.21.9 3.4.21.53 3.4.21.6 3.4.21.75 3.4.24.3 3.4.21.10 3.4.21.54 3.4.21.7 3.4.21.4 3.4.21.59 3.4.21.38 3.4.21.5 3.4.21.35 3.4.21.79 3.4.21.36 3.4.19.1 3.4.21.62 3.4.21.41 3.4.21.61 3.4.21.83 3.4.21.22 3.4.21.88 3.4.21.45 3.4.21.89 3.4.21.20 3.4.21.42 3.4.21.21 3.4.21.43 3.4.21.87 3.4.21.26 3.4.21.48 3.4.24.34 3.4.21.27 3.4.21.46 3.4.21.68 3.4.21.47 3.4.21.69 3.4.21.39 3.4.24.7 3.4.21.102 TYSND1 cleaves HSD17B4 The trypsin-like serine-type endopeptidase TYSND1 cleaves HSD17B4 (Peroxisomal multifunctional enzyme type 2, MFP-2) between amino acid residues 311 and 312, yielding HSD17B4(1-311) and HSD17B4(312-736) (Okumoto et al. 2011, also inferred from mouse homologs). HSD17B4(1-311) dehydrogenates 3-hydroxyacyl-CoA; HSD17B4(312-736) acts as an enoyl-CoA hydratase (Jiang et al. 1997, reviewed in Huyghe et al. 2006). (In vitro, HSD17B4(312-736) facilitates the transfer of 7-dehydrocholesterol and phosphatidylcholine between membranes.) The uncleaved protein (HSD17B4) also catalyzes these reactions. Authored: May, Bruce, 2017-12-22 Reviewed: Fransen, Marc, 2018-02-13 Reviewed: Van Veldhoven, Paul P, 2018-02-13 Reviewed: Azevedo, Jorge E, 2018-03-12 Edited: May, Bruce, 2017-12-22 DHB4_HUMAN HSD17B4(1-311) Peroxisomal multifunctional enzyme type 2 Reactome DB_ID: 9033503 1 EQUAL 311 EQUAL ReactomeDatabase ID Release 77 9033503 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033503 Reactome R-HSA-9033503 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033503.1 DHB4_HUMAN HSD17B4(312-736) Peroxisomal multifunctional enzyme type 2 Reactome DB_ID: 9033495 312 EQUAL 736 EQUAL ReactomeDatabase ID Release 77 9033495 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033495 Reactome R-HSA-9033495 1 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033495.1 ACTIVATION ReactomeDatabase ID Release 77 9033530 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033530 Reactome R-HSA-9033530 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033530.3 16766224 Pubmed 2006 Peroxisomal multifunctional protein-2: the enzyme, the patients and the knockout mouse model Huyghe, S Mannaerts, Guy P Baes, Myriam Van Veldhoven, Paul P Biochim Biophys Acta 1761:973-94 9089413 Pubmed 1997 Structure of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein Jiang, LL Miyazawa, S Souri, M Hashimoto, Takashi J Biochem 121:364-9 ReactomeDatabase ID Release 77 9033500 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033500 Reactome R-HSA-9033500 2 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033500.2 18952862 Pubmed 2008 The DEG15 serine protease cleaves peroxisomal targeting signal 2-containing proteins in Arabidopsis Schuhmann, Holger Huesgen, Pitter F Gietl, Christine Adamska, Iwona Plant Physiol. 148:1847-56 23459139 Pubmed 2013 Tysnd1 deficiency in mice interferes with the peroxisomal localization of PTS2 enzymes, causing lipid metabolic abnormalities and male infertility Mizuno, Yumi Ninomiya, Yuichi Nakachi, Yutaka Iseki, Mioko Iwasa, Hiroyasu Akita, Masumi Tsukui, Tohru Shimozawa, Nobuyuki Ito, Chizuru Toshimori, Kiyotaka Nishimukai, Megumi Hara, Hiroshi Maeba, Ryouta Okazaki, Tomoki Alodaib, Ali Nasser Ali Al Amoudi, Mohammed Jacob, Minnie Alkuraya, Fowzan S Horai, Yasushi Watanabe, Mitsuhiro Motegi, Hiromi Wakana, Shigeharu Noda, Tetsuo Kurochkin, Igor V Mizuno, Yosuke Schönbach, Christian Okazaki, Yasushi PLoS Genet. 9:e1003286 GENE ONTOLOGY GO:0016485 gene ontology term for cellular process MI MI:0359 ReactomeDatabase ID Release 77 9033241 数据库标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9033241 Reactome R-HSA-9033241 3 Reactome稳定的标识符。使用这个URL连接到the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-9033241.3 23798008 Pubmed 2013 Peroxisome biogenesis disorders: Biological, clinical and pathophysiological perspectives Braverman, Nancy E D'Agostino, Maria Daniela Maclean, Gillian E Dev Disabil Res Rev 17:187-96 16007078 Pubmed 2005 Functional role of the AAA peroxins in dislocation of the cycling PTS1 receptor back to the cytosol Platta, Harald W Grunau, Silke Rosenkranz, Katja Girzalsky, Wolfgang Erdmann, Ralf Nat. 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