glucocerebroside


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glucocerebroside

 [gloo″ko-ser´ĕ-bro-sīd]
a cerebroside containing a glucose sugar; it accumulates in the tissues in Gaucher's disease.

glu·co·syl·cer·a·mide

(glū'kō-sil-ser'ă-mīd),
A neutral glycolipid containing equimolar amounts of fatty acid, glucose, and sphingosine (or a derivative thereof); accumulates in people with Gaucher disease.
Synonym(s): glucocerebroside

glu·co·syl·cer·a·mide

(glū'kō-sil-ser'ă-mīd)
A neutral glycolipid containing equimolar amounts of fatty acid, glucose, and sphingosine (or a derivative thereof); accumulates in people with Gaucher disease.
Synonym(s): glucocerebroside.

Glucocerebroside

A cerebroside that contains glucose in the molecule.
Mentioned in: Gaucher Disease
References in periodicals archive ?
The clinical diagnosis of GD currently relies on demonstration of a deficient lysosomal enzyme acid [sz]-glucosidase (glucocerebrosidase [GBA]) which is responsible for the hydrolysis of glucocerebroside (glucosylceramide) into glucose and ceramide.[sup][3],[4] GBA gene is the only gene in which mutations are known to cause the GD.
This led to the accumulation of glucocerebroside in the phagocyte system, which led to the formation of Gaucher cells, induced other manifestations of GD, and eventually led to GD.
An alternative form of therapy is substrate reduction; medication with miglustat (Zavesca) reduces the amount of glucocerebroside, allowing the patient's depleted residual glucocerebrosidase activity to cope with the reduced amount of substrate.
(1) It is an autosomal recessive disorder resulting in an accumulation of glucocerebroside within cells of the reticuloendothelial system (spleen, liver, and bone marrow) as well as the lymph nodes, tonsils, and thymus.
Because individuals with Gaucher disease cannot break down glucocerebroside, it remains stored in some cells of the body.
Effect of various glycolipids on cAMP-kinase activity Effector Kinase activity (pmol/min) None 2.5 [+ or -] 0.2 SGL-II 63.0 [+ or -] 2.0 HF-SGL-II 59.0 [+ or -] 2.6 SGL-I 62.3 [+ or -] 1.0 SGL-I' 55.7 [+ or -] 3.0 FGL-V 49.1 [+ or -] 1.1 F-21 43.7 [+ or -] 1.8 Glucocerebroside 2.6 [+ or -] 0.1 cAMP 111.6 [+ or -] 3.7
Clinical trials for Niemann-Pick B [acid sphingomyelinase deficiency (ASM)] have recently been initiated, along with trials of alternate recombinant forms of glucocerebroside (for Gaucher disease).
Prevalence of glucocerebroside mutations in the Israeli Ashkenazi Jewish population.
(1973) Enzymatic synthesis of glucocerebroside by a glucosyltransferase from embryonic chicken brain.
Replacement therapy for inherited enzyme deficiency-macrophage-targeted glucocerebroside for Gaucher's disease.
We synthesized a series of fluorescent glucocerebrosides that contained pyrene-labeled fatty acids containing 3-11 methylene units.
Gaucher disease is an autosomal recessive lysosomal disorder secondary to lack of the enzyme glucocerebrosidase, leading to the accumulation of glucocerebrosides in the cells of the reticuloendothelial system, causing hepatosplenomegaly.