What does the glucocerebrosidase enzyme do?
Beta-glucocerebrosidase is a housekeeping enzyme that helps break down a large molecule called glucocerebroside into a sugar (glucose) and a simpler fat molecule (ceramide). Glucocerebroside is a component of the membrane that surrounds cells.
What is the substrate for glucocerebrosidase?
4MU-β-glc is the substrate most commonly used for the glucocerebrosidase enzyme assay, as well as for assaying many other lysosomal hydrolases.
What type of reaction does glucocerebrosidase catalyze?
Glucocerebrosidase is a lysosomal enzyme that is deficient or defective in the inherited condition known as Gaucher disease. The enzyme acts in lysosomes upon the sphingolipid glucocerebroside, catalyzing its conversion to glucose and ceramide.
What is the relationship between glucocerebrosidase and glycolipid?
Glucocerebrosidase (GCase) is a lysosomal glycoside hydrolase that cleaves the glycolipid glucosylceramide (GlcCer). Deficiencies of this enzyme lead to accumulation of GlcCer and the development of the lysosomal storage disease known as Gaucher’s disease.
Is glucocerebrosidase and Glucosylceramide the same thing?
Abstract: Glucocerebrosidase (GCase) is a retaining β-glucosidase with acid pH optimum metabolizing the glycosphingolipid glucosylceramide (GlcCer) to ceramide and glucose. Inherited deficiency of GCase causes the lysosomal storage disorder named Gaucher disease (GD).
Where does Glucocerebroside come from?
This recyclable material comes mainly from the breakdown of old red and white blood cells. In the brain, glucocerebroside comes from the processing of lipids during brain development and the formation of the myelin sheath (the fatty coating around each nerve fiber).
Where is glucocerebrosidase synthesized?
synthesis of an enzyme called glucocerebrosidase, leading to the accumulation of lipids called glucocerebrosides in Gaucher cells. Gaucher cells are large, wrinkled-appearing cells that store glycolipids and are usually found in the bone marrow and the spleen. The defective enzyme is glucocerebrosidase.
Is Gaucher disease a metabolic disease?
Gaucher disease is a rare, inherited metabolic disorder in which deficiency of the enzyme glucocerebrosidase results in the accumulation of harmful quantities of certain fats (lipids), specifically the glycolipid glucocerebroside, throughout the body especially within the bone marrow, spleen and liver.
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