Recombinant Human N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase (B3GNT1), Truncated

Uniprot Id

O43505

Target Species

Human

Target Name

B4GAT1

Target Full Name

Beta-1,4-glucuronyltransferase 1

Target Function

Beta-1,4-glucuronyltransferase involved in O-mannosylation of alpha-dystroglycan (DAG1). Transfers a glucuronic acid (GlcA) residue onto a xylose (Xyl) acceptor to produce the glucuronyl-beta-1,4-xylose-beta disaccharide primer, which is further elongated by LARGE1, during synthesis of phosphorylated O-mannosyl glycan. Phosphorylated O-mannosyl glycan is a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity. Required for axon guidance; via its function in O-mannosylation of alpha-dystroglycan (DAG1).

Target Involvement

Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A13 (MDDGA13)

Target Subcellular Location

Golgi apparatus membrane; Single-pass type II membrane protein.

Target Protein Families

Glycosyltransferase 49 family

Target Tissue Specificity

In the adult, highly expressed in heart, brain, skeletal muscle and kidney and to a lesser extent in placenta, pancreas, spleen, prostate, testis, ovary, small intestine and colon. Very weak expression in lung, liver, thymus and peripheral blood leukocyte

Target Synonyms

B4GAT1; B3GNT1; B3GNT6; Beta-1,4-glucuronyltransferase 1; I-beta-1,3-N-acetylglucosaminyltransferase; iGnT; N-acetyllactosaminide beta-1,3-N-acetylglucosaminyltransferase; Poly-N-acetyllactosamine extension enzyme; UDP-GlcNAc:betaGal beta-1,3-N-acetylglucosaminyltransferase 1