Description
Galactocerebrosidase (GALC) is also known as GALCERase, Galactocerebroside beta-galactosidase, Galactosylceramidase and Galactosylceramide beta-galactosidase, which belongs to the glycosyl hydrolase 59 family. Galactocerebrosidase / GALC hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. GALC with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon. Defects in Galc are the cause of the "twitcher" phenotype