glu·co·syl·cer·am·i·dase (gloo″ko-səl-sə-ramґĭ-dās) [EC] an enzyme of the hydrolase class that catalyzes the hydrolytic cleavage of glucose from glucocerebrosides to form ceramides, a reaction occurring in the lysosomal degradation of sphingolipids. Deficiency of enzyme activity, an autosomal recessive trait, results in Gaucher disease. Called also glucocerebrosidase.

Medical dictionary. 2011.

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  • Glucosylceramidase — Pfam box Symbol = Glyco hydro 30 Name = O Glycosyl hydrolase family 30 width = caption = Pfam= PF02055 InterPro= IPR001139 SMART= Prosite = SCOP = 1nof TCDB = OPM family= OPM protein= 1ogs PDB=PDB3|1y7vA:40 533 PDB3|1ogsA:40 533 PDB3|1nofA:31… …   Wikipedia

  • glucosylceramidase — noun Glucocerebrosidase …   Wiktionary

  • Gaucher's disease — Classification and external resources Acid beta glucosidase ICD 10 E75 …   Wikipedia

  • Glucocerebrosidase — Bändermodell des GBA Tetramer, nach PDB  …   Deutsch Wikipedia

  • Glucocerebrosidase — NOTOC Glucocerebrosidase (also called glucosylceramidase, β glucosidase, or D glucosyl N acylsphingosine glucohydrolase) is an enzyme (EC number| that is needed to cleave, by hydrolysis, the beta glucosidic linkage of the chemical… …   Wikipedia

  • Glukocerebrosidase — Glucocerebrosidase Vorhandene Strukturdaten: 1ogs …   Deutsch Wikipedia

  • Maladie De Gaucher — Autre nom Déficit en glucocérébrosidase Référence MIM …   Wikipédia en Français

  • Maladie de Gaucher — Référence MIM 230800 230900 231000 Transmission Récessive Chromosome 1q21 …   Wikipédia en Français

  • Maladie de gaucher — Autre nom Déficit en glucocérébrosidase Référence MIM …   Wikipédia en Français

  • imiglucerase — im·i·glu·cer·ase (im″ĭ glooґsər ās) an analogue of glucosylceramidase produced by recombinant DNA technology, used as an enzyme replenisher to replace glucosylceramidase (glucocerebrosidase) in type 1 Gaucher disease;… …   Medical dictionary