Alpha-Galactosidase (Gal; EC 3.2.1.22) hydrolyzes alpha-galactosyl moieties found in glycolipids and glycoproteins. In mammals, Gal hydrolyzes poly- and oligosaccharides commonly found in dietary sources that are difficult to digest. Therefore, 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 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 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. Assay Genie's Alpha Galactosidase Activity Assay Kit provides a simple, rapid way to monitor total Gal activity in wide variety of biological samples. In this kit, 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 Galactosidase activity.

Figure: (a) 4-Methylumbelliferon Standard Curve. Results are from multiple experiments. (b) α-Galactosidase Activity in Mouse Kidney Tissue Extracts (1 µg protein) and U937 Cell Lysates (0.2 µg protein). (c) Measurement of α-Galactosidase Activity in undiluted Human Pooled Saliva (5 µl). All assays were performed following kit protocol.