sucrase-isomaltase deficiency
- sucrase-isomaltase deficiency
- a disaccharidase deficiency caused by mutations in the SI gene (locus: 3q25-q26), which encodes the sucrase-isomaltase complex of the intestinal mucosa, leading to deficient activity of the complex with resulting malabsorption of sucrose and starch dextrins; it is characterized by watery, osmotic-fermentative diarrhea, sometimes leading to dehydration and malnutrition, manifest in infancy (congenital sucrose intolerance). While sucrase activity is always absent, α-dextrinase (isomaltase) activity may be either greatly reduced or relatively normal. See also disaccharide intolerance, under intolerance.
Medical dictionary.
2011.
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sucrase-isomaltase deficiency — suc·rase iso·mal·tase de·fi·cien·cy (sooґkrās i″so mawlґtās) see under deficiency … Medical dictionary
sucrase-isomaltase complex — the enzyme complex comprising sucrase and α dextrinase (isomaltase) activities, occurring in the brush border of the intestinal mucosa and hydrolyzing maltose as well as maltotriose and some other glycosidic bonds; the enzymes are… … Medical dictionary
Sucrase-isomaltase — protein Name=sucrase isomaltase (alpha glucosidase) caption= width= HGNCid=10856 Symbol=SI AltSymbols= EntrezGene=6476 OMIM=609845 RefSeq=NM 001041 UniProt=P14410 PDB= ECnumber=3.2.1.10 Chromosome=3 Arm=q Band=25.2 LocusSupplementaryData=… … Wikipedia
Sucrase — is the name given to a number of enzymes that catalyze the hydrolysis of sucrose to fructose and glucose. The enzyme invertase, which occurs more commonly in plants, also hydrolyzes sucrose but by a different mechanism.[1] Contents 1 Types 2 … Wikipedia
sucrase — SYN: sucrose α d glucohydrolase. * * * su·crase sü .krās, .krāz n INVERTASE * * * n. an enzyme, secreted by glands in the small intestine, that catalyses the hydrolysis of sucrose into its components (glucose and fructose). * * * su·crase… … Medical dictionary
disaccharidase deficiency — less than normal activity of disaccharidases of the intestinal mucosa. It usually denotes a generalized deficiency of all such enzymes secondary to a disorder of the small intestine, which clinically may be manifest only as a deficiency of… … Medical dictionary
congenital sucrose-isomaltose malabsorption — sucrase isomaltase deficiency … Medical dictionary
Sucrose intolerance — Classification and external resources Sucrose ICD 10 E … Wikipedia
congenital sucrose intolerance — a disaccharide intolerance specific for sucrose, usually due to a congenital defect in the sucrase isomaltase enzyme complex; see sucrase isomaltase deficiency, under deficiency … Medical dictionary
α-dextrinase — α dex·trin·ase (dekґstrin ās) an enzyme that catalyzes the hydrolysis of both α 1,6 and α 1,4 bonds in linear and branched oligoglucosides and maltose and isomaltose. It occurs on the brush border of the intestinal mucosa,… … Medical dictionary
