Alpha-Galactosidase (a-Gal; EC 3.2.1.22) hydrolyzes alpha-galactosyl moieties found in glycolipids and glycoproteins. In mammals, a-Gal hydrolyzes poly- and oligosaccharides commonly found in dietary sources that are difficult to digest. Therefore, a-Gal is used in dietary supplements that help to reduce the production of intestinal gases due to consumption of certain foods. It is known total a-Gal activity is due to two major isozymes with unique, yet different thermostability profiles. Alpha-Galactosidase A, is thermolabile and represents approximately 90% of total a-Gal activity. Fabry Disease, a lysosomal disease disorder, is characterized by mutations in alpha-Galactosidase A. These mutations cause abnormal accumulation of glycosphingolipids in lysosomes. The Alpha Galactosidase Activity Assay Kit provides a simple, rapid way to monitor total a-Gal activity in wide variety of biological samples. In this kit, a-Gal cleaves a synthetic specific substrate releasing a fluorophore, which can be easily quantified (Ex/Em= 360/445 nm). The assay is specific, sensitive and can detect as low as 0.1 μU of a-Galactosidase activity. Sample types: biological fluids, cell lysates, kidney homogenate, saliva, tissue homogenates, u937
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Applications
Measurement of a-Galactosidase activity in various samples