• β-Glucocerebrosidase (also called acid β-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, or GCase) is an enzyme with glucosylceramidase activity (EC 3.2.1.45) that cleaves by hydrolysis the β-glycosidic linkage of the chemical glucocerebroside, an intermediate in glycolipid metabolism that is abundant in cell membranes (particularly skin cells). (wikipedia.org)
  • The GBA gene provides instructions for making an enzyme called beta-glucocerebrosidase. (medlineplus.gov)
  • Variants in the GBA gene greatly reduce or eliminate the activity of beta-glucocerebrosidase. (medlineplus.gov)
  • The disorder results from the deficiency of a specific lysosomal hydrolase, glucocerebrosidase (also termed acid beta-glucosidase, glucosylceramidase). (medscape.com)
  • The enzyme lysosomal glucocerebrosidase (beta-glucosidase, glucosylceramidase)catalyzes the breakdown of a cell membrane constituent of red and white blood cells, called glucosylceramide. (tu-muenchen.de)
  • The cause of Gaucher disease is a recessive mutation in a houskeeping gene lysosomal glucocerebrosidase (beta-glucosidase, glucosylceramidase) on chromosome 1 (1q21). (tu-muenchen.de)
  • A beta-Glucosidase ( GBA) gene defect results in glucocerebrosidase enzyme deficiency. (biomedcentral.com)
  • beta-Glucocerebrosidase 2 N terminal. (unl.edu)
  • This domain is found in the protein beta-Glucocerebrosidase 2. (unl.edu)
  • Glucocerebrosidase/GBA (also called acid β-glucosidase, Glucosylceramidase) is a lysosomal enzyme responsible for the breakdown of glucocerebroside releasing glucose and ceramide. (anaspec.com)
  • The biochemical investigation implicated measuring plasma chitotriosidase enzyme activity followed by confirmatory test of β-Glucosidase enzyme activity from the leukocytes. (biomedcentral.com)
  • Under normal condition, a lysosomal enzyme β-glucosidase (EC 3.2.1.45) catabolize glucocerebroside to glucose and ceramide [ 5 ]. (biomedcentral.com)
  • A debranching enzyme is a molecule that helps facilitate the breakdown of glycogen , which serves as a store of glucose in the body, through glucosyltransferase and glucosidase activity. (wikidoc.org)
  • Glucosyltransferase and glucosidase are performed by a single enzyme in mammals, yeast, and some bacteria, but by two distinct enzymes in E. coli and other bacteria, complicating nomenclature. (wikidoc.org)
  • When glucosyltransferase and glucosidase are catalyzed by distinct enzymes, "glycogen debranching enzyme" usually refers to the glucosidase enzyme . (wikidoc.org)
  • Gaucher disease: A G+1----A+1 IVS2 splice donor site mutation causing exon 2 skipping in the acid beta-glucosidase mRNA. (nih.gov)
  • An autosomal recessive disorder caused by a deficiency of acid beta-glucosidase (GLUCOSYLCERAMIDASE) leading to intralysosomal accumulation of glycosylceramide mainly in cells of the MONONUCLEAR PHAGOCYTE SYSTEM. (umassmed.edu)
  • Acid beta-glucosidase 1 counteracts p38delta-dependent induction of interleukin-6: possible role for ceramide as an anti-inflammatory lipid. (musc.edu)
  • In the majority of organisms, fatty acid degradation occurs mostly through the beta-oxidation cycle. (smpdb.ca)
  • Saposin-A and saposin-C stimulate the hydrolysis of glucosylceramide by beta-glucosylceramidase (EC 3.2.1.45) and galactosylceramide by beta-galactosylceramidase (EC 3.2.1.46). (affbiotech.cn)
  • We developed and applied fluorescent activity-based probes reporting on either human GH30 lysosomal glucosylceramidase (GBA1, a retaining β-glucosidase) or GH31 lysosomal retaining α-glucosidase (GAA). (hal.science)
  • Saposin-B stimulates the hydrolysis of galacto-cerebroside sulfate by arylsulfatase A (EC 3.1.6.8), GM1 gangliosides by beta-galactosidase (EC 3.2.1.23) and globotriaosylceramide by alpha-galactosidase A (EC 3.2.1.22). (affbiotech.cn)
  • Amylo-α-1,6-glucosidase ( EC 3.2.1.33 ), or glucosidase , cleaves the remaining alpha-1,6 linkage, producing glucose and a linear chain of glycogen. (wikidoc.org)
  • [5] The mechanism by which the glucosidase cleaves the α -1,6-linkage is not fully known because the amino acids in the active site have not yet been identified. (wikidoc.org)
  • The biochemical analysis revealed a significant reduction in the β-Glucosidase activity in all the patients. (biomedcentral.com)
  • Thus the debranching enzymes, transferase and α-1,6- glucosidase converts the branched glycogen structure into a linear one, paving the way for further cleavage by phosphorylase. (wikidoc.org)
  • In E. coli and other bacteria, glucosyltransferase and glucosidase functions are performed by two distinct enzymes. (wikidoc.org)
  • All participants were evaluated with serum calcium, 25-hydroxy vitamin D, parathyroid hormone, N-terminal propeptide of type 1 collagen (P1NP) and Beta-CrossLaps (CTx) and BMD at LS and FN using dual X-ray absorptiometry (DXA). (bvsalud.org)
  • beta-glucosidase accumulates in the brain because of the turnover of complex lipids during brain development and formation of the myelin envelope of nerve cells. (tu-muenchen.de)
  • Gamma-crystallins are similar in structure to BETA-CRYSTALLINS in that they both form into a Greek key-like structure. (lookformedical.com)
  • Alpha, beta, and delta crystallins occur in avian and reptilian lenses, while alpha, beta, and gamma crystallins occur in all other lenses. (lookformedical.com)
  • This graph shows the total number of publications written about "Glucosidases" by people in this website by year, and whether "Glucosidases" was a major or minor topic of these publications. (jefferson.edu)
  • Beta crystallin a chain. (lookformedical.com)
  • Beta-crystallins exist as oligomers formed from acidic (BETA-CRYSTALLIN A CHAIN) and basic (BETA-CRYSTALLIN B CHAIN) subunits. (lookformedical.com)
  • Non-lysosomal glucosylceramidase that catalyzes the conversion of glucosylceramide (GlcCer) to free glucose and ceramide. (nih.gov)
  • The catalytic domain consists of a (beta/alpha)(8) TIM barrel, as expected for a member of the glucosidase hydrolase A clan. (proteopedia.org)