BioPAX pathway converted from "arginine + H2O => ornithine + urea [ARG1]" in the Reactome database.
3.5.3.1
精氨酸+水= > ornithine + urea [ARG1]
精氨酸+水= > ornithine + urea [ARG1]
Cytosolic Arginase 1 (ARG1) trimer catalyzes the hydrolysis of arginine to yield ornithine and urea (DiCostanzo et al. 2005). Patients expressing mutated forms of the enzyme with diminished in vitro arginase activity can accumulate arginine to pathogenic levels in the blood (e.g., Uchino et al. 1995).
Authored: D'Eustachio, P, 2003-06-24 00:00:00
Edited: D'Eustachio, P, 2003-06-24 00:00:00
Reactome DB_ID: 29356
1
cytosol
GO
0005829
water [ChEBI:15377]
water
Reactome
//www.joaskin.com
ChEBI
15377
Reactome DB_ID: 29468
1
L-argininium(1+) [ChEBI:32682]
L-argininium(1+)
ODKSFYDXXFIFQN-BYPYZUCNSA-O
InChI=1S/C6H14N4O2/c7-4(5(11)12)2-1-3-10-6(8)9/h4H,1-3,7H2,(H,11,12)(H4,8,9,10)/p+1/t4-/m0/s1
L-arginine monocation
L-argininium
C6H15N4O2
(2S)-2-ammonio-5-guanidiniopentanoate
175.20906
L-arginine
NC(=[NH2+])NCCC[C@H]([NH3+])C([O-])=O
ChEBI
32682
Reactome DB_ID: 29514
1
尿素(ChEBI: 16199)
urea
ChEBI
16199
Reactome DB_ID: 29498
1
L-ornithine [ChEBI:15729]
L-ornithine
ChEBI
15729
PHYSIOL-LEFT-TO-RIGHT
ACTIVATION
Reactome DB_ID: 70567
ARG1 trimer [cytosol]
ARG1 trimer
arginase 1 homotrimer
Reactome DB_ID: 70561
3
UniProt:P05089 ARG1
ARG1
ARG1
FUNCTION Key element of the urea cycle converting L-arginine to urea and L-ornithine, which is further metabolized into metabolites proline and polyamides that drive collagen synthesis and bioenergetic pathways critical for cell proliferation, respectively; the urea cycle takes place primarily in the liver and, to a lesser extent, in the kidneys.FUNCTION Functions in L-arginine homeostasis in nonhepatic tissues characterized by the competition between nitric oxide synthase (NOS) and arginase for the available intracellular substrate arginine. Arginine metabolism is a critical regulator of innate and adaptive immune responses. Involved in an antimicrobial effector pathway in polymorphonuclear granulocytes (PMN). Upon PMN cell death is liberated from the phagolysosome and depletes arginine in the microenvironment leading to suppressed T cell and natural killer (NK) cell proliferation and cytokine secretion (PubMed:15546957, PubMed:16709924, PubMed:19380772). In group 2 innate lymphoid cells (ILC2s) promotes acute type 2 inflammation in the lung and is involved in optimal ILC2 proliferation but not survival (By similarity). In humans, the immunological role in the monocytic/macrophage/dendritic cell (DC) lineage is unsure.PATHWAY Nitrogen metabolism; urea cycle; L-ornithine and urea from L-arginine: step 1/1.SUBUNIT Homotrimer (PubMed:16141327, PubMed:17469833, PubMed:17562323, PubMed:18802628, PubMed:2241902). Interacts with CMTM6 (PubMed:28813417).TISSUE SPECIFICITY Within the immune system initially reported to be selectively expressed in granulocytes (polymorphonuclear leukocytes [PMNs]) (PubMed:15546957). Also detected in macrophages mycobacterial granulomas (PubMed:23749634). Expressed in group2 innate lymphoid cells (ILC2s) during lung disease (PubMed:27043409).INDUCTION By arginine or homoarginine.SIMILARITY Belongs to the arginase family.
Homo sapiens
NCBI Taxonomy
9606
UniProt
P05089
Chain Coordinates
1
EQUAL
322
EQUAL
Reactome DB_ID: 29418
6
manganese(2+) [ChEBI:29035]
manganese(2+)
manganese(II)
manganous ion
MANGANESE (II) ION
manganese, ion (Mn2+)
Mn(2+)
ChEBI
29035
Reactome Database ID Release 77
70567
Database identifier. Use this URL to connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=70567
Reactome
R-HSA-70567
1
Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-70567.1
GO
0004053
GO molecular function
Reactome Database ID Release 77
70568
Database identifier. Use this URL to connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=70568
Reactome Database ID Release 77
70569
Database identifier. Use this URL to connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=70569
Reactome
R-HSA-70569
3
Reactome stable identifier. Use this URL to connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-70569.3
16141327
Pubmed
2005
Crystal structure of human arginase I at 1.29-A resolution and exploration of inhibition in the immune response
Di Costanzo, L
Sabio, G
Mora, A
Rodriguez, PC
Ochoa, AC
Centeno, F
Christianson, DW
Proc Natl Acad Sci U S A 102:13058-63
7649538
Pubmed
1995
Molecular basis of phenotypic variation in patients with argininemia
Uchino, T
Snyderman, SE
Lambert, M
Qureshi, IA
Shapira, SK
Sansaricq, C
Smit, LM
Jakobs, Cornelis
Matsuda, I
Hum Genet 96:255-60