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Alpha-galactosidase

Exoglycosidases are enzymes which can modify cell membrane carbohydrate epitopes and thereby modify the immune response. Alpha-galactosidase is a well-known enzyme involved in the hydrolysis of alpha-galactosides present in, for instance, various important plants or plant parts used for nutritional purposes such as legumes, vegetables, grains, cereals and the like. The alpha-galactosidase A enzyme is a lysosomal enzyme which hydrolyzes globotriaosylceramide and related glycolipids which have terminal alpha-galactosidase linkages. The enzyme alpha-galactosidase (alpha-D-galactoside galactohydrolase) catalyzes the hydrolysis of the terminal linked alpha-galactose moiety from galactose-containing oligosaccharides. These include, for example, the naturally occurring disaccharide melibiose, the trisaccharide raffinose and the tetrasaccharide stachyose. Alpha-galactosidases have potential use in various applications. Fabry disease is an X-linked inborn error of metabolism resulting from a deficiency of the lysosomal enzyme, alpha-galactosidase A. Deficiency of alpha-galactosidase A results in the accumulation of its major glycosphingolipid substrate, globotriaosylceramide and related glycolipids with terminal alpha-galactosidic linkages. Progressive substrate deposition, especially in the plasma and vascular endothelium, leads to ischemia arid infarction with early demise due to vascular disease of the heart and kidney. Alpha-galactosidase enzymes are produced by various microorganisms, plants and animals. Mammals, however, are deficient in intestinal alpha-galactosidase production and, consequently, are incapable of decomposing ingested alpha-galactosides by themselves.

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