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Glucosylceramidase

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Definition: A glycosidase that hydrolyzes a glucosylceramide to yield free ceramide plus glucose. Deficiency of this enzyme leads to abnormally high concentrations of glucosylceramide in the brain in GAUCHER DISEASE. EC 3.2.1.45.  /defic: consider also GAUCHER DISEASE   
See Also Gaucher Disease 		Other names Glucocerebrosidase; beta-Glucosidase, Glucosylceramide; beta-Glucosidase, Glucocerebroside; beta-Glucosidase, Acid; beta Glucocerebrosidase; Hydrolase, Glucosylsphingosine Glucosyl; Glucosylceramide beta Glucosidase; Glucosyl Hydrolase, Glucosylsphingosine; Glucocerebroside beta Glucosidase; Ceramidase, Glucosyl; Acid beta Glucosidase; beta-Glucocerebrosidase; Glucosylsphingosine Glucosyl Hydrolase; Glucosylceramide beta-Glucosidase; Glucosyl Ceramidase; Glucocerebroside beta-Glucosidase; Acid beta-GlucosidaseDrugs* : ELELYSO
 
SubstanceCAS Registry & nameCategoriesSourceDrugs*
Velaglucerase alfa, human  EC 3.2.1.45   *Glucosylceramidase. Curr Opin Investig Drugs. 2010 Apr;11(4):472-8VPRIV
N-octyl-beta-valienamine  0   *Hexosamines Glucosylceramidase/antagonists & inhibitors. Bioorg Med Chem 1998 Oct;6(10):1955-62
imiglucerase  EC 3.2.1.-   *Glucosylceramidase Gaucher Disease Recombinant Proteins. Ann Intern Med 1995 Jan 1;122(1):33-9CEREZYME
bromoconduritol-B-epoxide  24579-86-0   *Epoxy Compounds Inositol/*analogs & derivatives Glucosylceramidase/antagonists & inhibitors. J Lipid Res 1994 May;35(5):905-12
alglucerase  EC 3.2.1.-   *Glucosylceramidase Gaucher Disease Enzyme Replacement Therapy. Med Lett Drugs Ther 1991;33(851):82CEREDASE
2-deoxy-2-fluoroglucopyranosyl fluoride  85754-73-0   Deoxyglucose/*analogs & derivatives Glucosylceramidase/antagonists & inhibitors. Eur J Nucl Med 1984;9(2):77
castanospermine  79831-76-8   *Indolizines alpha-Glucosidases/antagonists & inhibitors beta-Glucosidase/antagonists & inhibitors Glucosylceramidase/antagonists & inhibitors Stereoisomerism.
Pharma Action Anti-Inflammatory Agents, Non-Steroidal ; Enzyme Inhibitors ; Immunosuppressive Agents 		Arch Biochem Biophys 1983;221(2):593
6-bromoconduritol  42014-74-4 4-Cyclohexene-1,2,3-triol, 6-bromo  *Cyclohexenes Inositol/*analogs & derivatives beta-Glucosidase/antagonists & inhibitors Glucosylceramidase/antagonists & inhibitors. Proc Natl Acad Sci USA 1982;79(22):6787
conduritol epoxide  6090-95-5 1,2-anhydro-myo-inositol  Inositol/*analogs & derivatives beta-Glucosidase/antagonists & inhibitors Glucosylceramidase/antagonists & inhibitors.
Pharma Action Enzyme Inhibitors 		Arch Pathol Lab Med 101(5):255;1977; Biochem Biophys Acta 452(2):542;1976; Biochem Biophys Res Commun 67(1):85;1975; Biochim Biophys Acta 1979;571(1):70; Biochim Biophys Acta 483:141;1977; Biochim Biophys Acta 485:141;1977; Biochim Biophys Acta 582(3):412;1979; J Neurochem 30:1023;1978

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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