glucocerebroside


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glucocerebroside

[¦glü·kō·sə′rēb·rə‚sīd]
(biochemistry)
A glycoside of ceramide that contains glucose.
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Background: Gaucher's disease (GD) is an autosomal recessive disorder caused by a deficiency of acid [sz]-glucosidase (glucocerebrosidase [GBA]) that results in the accumulation of glucocerebroside within macrophages.
GD affects the ability of cells to break down a specific type of fat, glucocerebroside (GC), as a result of the deficiency or limited activity of the enzyme glucocerebrosidase (GCase).
Indeed, life may be tough for individuals who suffer from Gaucher's disease, a rare and inherited disorder wherein the deficiency of enzyme called beta-glucocerebrosidase (that is produced in our cells) results in the accumulation of a fatty substance called glucocerebroside into a sugar and a simpler fat molecule throughout the body, more often in the bone marrow, spleen and liver.
The decreased enzymatic activity leads to accumulation of glucocerebroside within characteristic "Gaucher cells" of mononuclear phagocyte origin identified in most tissues.
The striated appearance in Gaucher disease is due to the deposition of glucocerebroside, not immunoglobulins; therefore, the pale-staining striated inclusions in Gaucher cells lack the refractile and tinctorial characteristics of those in CSH.
Type I is a disorder caused by a lack of glucocerebrosidase, an enzyme that helps clear glucocerebroside from cellular structures called lysosomes.
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).
The resultant accumulation of the substrate glucocerebroside causes the organ damage.
Deficient GCase activity leads to lysosomal accumulation of glucocerebroside inside certain cells, which is believed to cause the various symptoms of Gaucher disease, including an enlarged liver and spleen, abnormally low levels of red blood cells and platelets and skeletal complications.
This results in the accumulation of glucocerebroside in the cells of the reticuloendothelial system, including the bone marrow.
Gaucher disease Type I is a disorder caused by a lack of glucocerebrosidase, an enzyme that helps clear glucocerebroside from cellular structures called lysosomes.
Gaucher disease is caused by a deficiency of the enzyme glucocerebrosidase, which is responsible for breaking down and digesting glucocerebroside, a by-product of the normal recycling of red blood cells.