Lysosomal glucosylceramidase beta also known as acid beta-glucosidase (E.C. 3.2.1.45) is a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide (GC). The enzyme plays a central role in the degradation of complex lipids and the turnover of cellular membranes. Through the production of ceramides, it participates in the PKC-activated salvage pathway of ceramide formation. Glucosylceramidase also plays a role in cholesterol metabolism. It may catalyze the glucosylation of cholesterol through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol. Deficiency of Glucosylceramidase results in the accumulation of GC in lysosomes. This leads to Gaucher disease, an inherited disorder that affects many organs and tissues. BioVision's Glucosylceramidase Activity Assay Kit provides a simple, rapid way to monitor Glucosylceramidase activity in a wide variety of biological samples. In this kit, Glucosylceramidase cleaves a specific synthetic substrate and releases a fluorophore, which can be easily quantified (Ex/Em=360/445 nm). The assay is specific, sensitive and can detect as low as 0.2 μU of Glucosylceramidase activity in variety of samples.
Size
100 assays
Concentration
n/a
Applications
Screening for Gaucher Disease, Measurement of Glucosylceramidase activity in various tissues/cells