InterPro domain: IPR026283

This entry represents a subset of the glycosyl hydrolase 35 family, including beta-galactosidase (B-gal), B-gal-1, B-gal-1-like, B-gal-8, B-gal-17. Mammalian beta-galactosidase is a lysosomal enzyme (gene GLB1) which cleaves the terminal galactose from gangliosides, glycoproteins, and glycosaminoglycans and whose deficiency is the cause of the genetic disease Gm(1) gangliosidosis (Morquio disease type B)[ 1 , 2 ].

1. Conserved catalytic machinery and the prediction of a common fold for several families of glycosyl hydrolases. Proc. Natl. Acad. Sci. U.S.A. 92, 7090-4
2. Structures and mechanisms of glycosyl hydrolases. Structure 3, 853-9