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