UDP Glycosyltransferase 8 (UGT8)

Galactocerebrosides are abundant sphingolipids of the myelin membrane of the central nervous system and peripheral nervous system and are also present in small amounts in kidney. The key enzymatic step in the biosynthesis of galactocerebrosides consists of the transfer of galactose to ceramide catalyzed by UDP-galactose ceramide galactosyltransferase (CGT). The enzyme encoded by the CGT gene is the first involved in complex lipid biosynthesis in the myelinating oligodendrocyte.The same part of the predicted amino acid sequence that binds the ceramide moiety binds aglycone in isozymes of the UGT subfamilies. Only subtle changes of the rather conserved C-terminal domain appeared to be sufficient to confer the specificity of the binding site for the activated sugar donor molecule UDP-galactose.