Beta-1,4-galactosyltransferase 2 (B4GALT2)

Beta-1,4-galactosyltransferase 2 (B4GALT2) is an enzyme responsible for transferring galactose in a beta-1,4 linkage to acceptor sugars (such as N-acetylglucosamine, glucose, and xylose) during the biosynthesis of glycoconjugates, especially N-acetyllactosamine in glycoproteins and glycolipids. It is a type II membrane glycoprotein localized primarily to the Golgi apparatus, where its N-terminal domain serves as a membrane anchor. B4GALT2 displays exclusive specificity for its donor substrate, UDP-galactose, and its substrate specificity can be modified by the presence of alpha-lactalbumin, though it is not active in lactating mammary tissue. This enzyme plays a crucial role in the construction of complex N-linked oligosaccharides and the carbohydrate moieties of glycolipids. Mutations in B4GALT2 are associated with Ehlers-Danlos syndrome, spondylodysplastic type 2. There are several transcript variants encoding different isoforms of the protein.

Other names

Beta4Gal-T2Beta-1,4-GalTase 2Beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferaseN-acetyllactosamine synthaseUDP-galactose:beta-N-acetylglucosamine beta-1,4-galactosyltransferase 2Lactose synthase A proteinNal synthaseBeta-N-acetylglucosaminyl-glycolipid beta-1,4-galactosyltransferaseUDP-Gal:betaGlcNAc beta 1,4-galactosyltransferase 2Beta-4-GalT2EC 2.4.1.38EC 2.4.1.22EC 2.4.1.90B4Gal-T3 (sometimes listed as a synonym, but refers to a distinct gene)