Symbol Glyco_hydro_59 Pfam clan CL0058 OPM superfamily 125 | Pfam PF02057 InterPro IPR001286 OPM protein 3zr5 | |
In molecular biology, glycoside hydrolase family 59 is a family of glycoside hydrolases.
Glycoside hydrolases EC 3.2.1. are a widespread group of enzymes that hydrolyse the glycosidic bond between two or more carbohydrates, or between a carbohydrate and a non-carbohydrate moiety. A classification system for glycoside hydrolases, based on sequence similarity, has led to the definition of >100 different families. This classification is available on the CAZy(http://www.cazy.org/GH1.html) web site, and also discussed at CAZypedia, an online encyclopedia of carbohydrate active enzymes.
Glycoside hydrolase family 59 CAZY GH_59 comprises enzymes with only one known activity; galactocerebrosidase (EC 3.2.1.46). Globoid cell leukodystrophy (Krabbe disease) is a severe, autosomal recessive disorder that results from deficiency of galactocerebrosidase (GALC) activity. GALC is responsible for the lysosomal catabolism of certain galactolipids, including galactosylceramide and psychosine.