Galactosylgalactosylxylosylprotein 3-Beta-Glucuronosyltransferase 3 (B3GAT3) Mouse Recombinant Protein

B3GAT3: Glycosaminoglycans biosynthesis. Involved in forming the linkage tetrasaccharide present in heparan sulfate and chondroitin sulfate. Transfers a glucuronic acid moiety from the uridine diphosphate-glucuronic acid (UDP-GlcUA) to the common linkage region trisaccharide Gal-beta-1,3-Gal-beta-1,4-Xyl covalently bound to a Ser residue at the glycosaminylglycan attachment site of proteoglycans. Can also play a role in the biosynthesis of l2/HNK-1 carbohydrate epitope on glycoproteins. Shows strict specificity for Gal-beta-1,3-Gal-beta-1,4-Xyl, exhibiting negligible incorporation into other galactoside substrates including Galbeta1-3Gal beta1-O-benzyl, Galbeta1-4GlcNAc and Galbeta1-4Glc. Defects in B3GAT3 are the cause of multiple joint dislocations short stature craniofacial dysmorphism and congenital heart defects (JDSSDHD). An autosomal recessive disease characterized by dysmorphic facies, bilateral dislocations of the elbows, hips, and knees, clubfeet, and short stature, as well as cardiovascular defects. Belongs to the glycosyltransferase 43 family.

Protein type: Membrane protein, integral; Transferase; Glycan Metabolism - heparan sulfate biosynthesis; EC 2.4.1.135; Glycan Metabolism - chondroitin sulfate biosynthesis

Cellular Component: Golgi apparatus; cis-Golgi network; membrane; integral to membrane

Molecular Function: transferase activity; glucuronosyltransferase activity; metal ion binding; galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity

Biological Process: positive regulation of catalytic activity; glycosaminoglycan biosynthetic process; heparan sulfate proteoglycan biosynthetic process; chondroitin sulfate proteoglycan biosynthetic process; dermatan sulfate proteoglycan biosynthetic process