BioPAX pathway converted from "Defective SI does not hydrolyze maltotriose" in the Reactome database.
LEFT-TO-RIGHT
3.2.1.20
Defective SI does not hydrolyze maltotriose
Mutations that disrupt the catalytic activity or strongly interfere with proper folding, glycosylation and transport of SI (sucrase-isomaltase) are inferred to block the cleavage of maltotriose to maltose and glucose, based on the experimentally demonstrated failure of these SI mutant proteins to hydrolyze maltose (e.g., Sander et al. 2005) and the broad substrate specificity of the normal enzyme (Sim et al. 2010).
Authored: D'Eustachio, Peter, 2015-02-15
Reviewed: Naim, Hassan Y, Amiri, Mahdi, 2015-02-15
Reviewed: Jassal, Bijay, 2015-01-29
Edited: D'Eustachio, Peter, 2015-02-15
H2O
water
Reactome DB_ID: 109276
extracellular region
GENE ONTOLOGY
GO:0005576
water [ChEBI:15377]
water
ChEBI
CHEBI:15377
Reactome Database ID Release 77
109276
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=109276
Reactome
R-ALL-109276
4
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-109276.4
Reactome
//www.joaskin.com
COMPOUND
C00001
additional information
MI
MI:0361
maltotriose
alpha-maltotriose
Reactome DB_ID: 191103
alpha-maltotriose [ChEBI:27931]
alpha-maltotriose
ChEBI
CHEBI:27931
Reactome Database ID Release 77
191103
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=191103
Reactome
R-ALL-191103
3
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-ALL-191103.3
ACTIVATION
Converted from EntitySet in Reactome
SI mutant dimers
Reactome DB_ID: 5659923
sucrase-isomaltase dimer C1229Y
Reactome DB_ID: 5659911
plasma membrane
GENE ONTOLOGY
GO:0005886
sucrase-isomaltase C1229Y
Reactome DB_ID: 5659912
SI(1-1006)
isomaltase fragment of sucrase-isomaltase
Reactome DB_ID: 189090
UniProt:P14410 SI
SI
FUNCTION Plays an important role in the final stage of carbohydrate digestion. Isomaltase activity is specific for both alpha-1,4- and alpha-1,6-oligosaccharides.SUBUNIT The resulting sucrase and isomaltase subunits stay associated with one another in a complex by non-covalent linkages.TISSUE SPECIFICITY Expressed in the poorly differentiated crypt cells of the small intestine as well as in the mature villous cells. Expressed at very low levels in the colon.PTM The precursor is proteolytically cleaved when exposed to pancreatic proteases in the intestinal lumen.PTM Sulfated.MISCELLANEOUS There is a high degree of homology between the isomaltase and sucrase portions (41% of amino acid identity) indicating that this protein is evolved by partial gene duplication.SIMILARITY Belongs to the glycosyl hydrolase 31 family.
Homo sapiens
NCBI Taxonomy
9606
UniProt
P14410
1
EQUAL
1006
EQUAL
Reactome Database ID Release 77
189090
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=189090
Reactome
R-HSA-189090
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-189090.1
1
SI(1007-1826) C1229Y
sucrase fragment of sucrase-isomaltase C1229Y
Reactome DB_ID: 5659913
1229
EQUAL
L-cysteine removal
MOD
MOD:01635
1007
EQUAL
1826
EQUAL
Reactome Database ID Release 77
5659913
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659913
Reactome
R-HSA-5659913
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659913.1
1
Reactome Database ID Release 77
5659912
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659912
Reactome
R-HSA-5659912
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659912.1
2
Reactome Database ID Release 77
5659911
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659911
Reactome
R-HSA-5659911
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659911.1
sucrase-isomaltase dimer Q1098P
Reactome DB_ID: 5659914
sucrase-isomaltase Q1098P
Reactome DB_ID: 5659906
1
SI(1007-1826) Q1098P
sucrase fragment of sucrase-isomaltase Q1098P
Reactome DB_ID: 5659901
1098
EQUAL
L-glutamine removal
MOD
MOD:01637
1007
EQUAL
1826
EQUAL
Reactome Database ID Release 77
5659901
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659901
Reactome
R-HSA-5659901
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659901.1
1
Reactome Database ID Release 77
5659906
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659906
Reactome
R-HSA-5659906
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659906.1
2
Reactome Database ID Release 77
5659914
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659914
Reactome
R-HSA-5659914
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659914.1
sucrase-isomaltase dimer Q117R
Reactome DB_ID: 5659887
sucrase-isomaltase Q117R
Reactome DB_ID: 5659888
SI(1-1006) Q117R
isomaltase fragment of sucrase-isomaltase Q117R
Reactome DB_ID: 5659882
117
EQUAL
1
EQUAL
1006
EQUAL
Reactome Database ID Release 77
5659882
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659882
Reactome
R-HSA-5659882
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659882.1
1
SI(1007-1826)
sucrase fragment of sucrase-isomaltase
Reactome DB_ID: 189035
1007
EQUAL
1826
EQUAL
Reactome Database ID Release 77
189035
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=189035
Reactome
R-HSA-189035
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-189035.1
1
Reactome Database ID Release 77
5659888
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659888
Reactome
R-HSA-5659888
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659888.1
2
Reactome Database ID Release 77
5659887
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659887
Reactome
R-HSA-5659887
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659887.1
Reactome Database ID Release 77
5659923
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659923
Reactome
R-HSA-5659923
1
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659923.1
GENE ONTOLOGY
GO:0004558
gene ontology term for cellular function
MI
MI:0355
Same Catalyst Activity
Reactome Database ID Release 77
9631884
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=9631884
Reactome Database ID Release 77
5659899
数据库标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser?DB=gk_current&ID=5659899
Reactome
R-HSA-5659899
2
Reactome稳定的标识符。使用这个URL connect to the web page of this instance in Reactome: //www.joaskin.com/cgi-bin/eventbrowser_st_id?ST_ID=R-HSA-5659899.2
20356844
Pubmed
2010
Structural basis for substrate selectivity in human maltase-glucoamylase and sucrase-isomaltase N-terminal domains
Sim, Lyann
Willemsma, Carly
Mohan, Sankar
Naim, Hassan Y
Pinto, B Mario
Rose, David R
J. Biol. Chem. 285:17763-70
16329100
Pubmed
2006
Novel mutations in the human sucrase-isomaltase gene (SI) that cause congenital carbohydrate malabsorption
Sander, Petra
Alfalah, Marwan
Keiser, Markus
Korponay-Szabo, Ilma
Kovács, Judit B
Leeb, Tosso
Naim, Hassan Y
Hum. Mutat. 27:119