Description
Aliases
Beta-1, 4-glucuronyltransferase 1, I-beta-1, 3-N-acetylglucosaminyltransferase, N-acetyllactosaminide beta-1, 3-N-acetylglucosaminyltransferase, Poly-N-acetyllactosamine extension enzyme, UDP-GlcNAc:betaGal beta-1, 3-N-acetylglucosaminyltransferase 1, B4GAT1, B3GNT1
Antibody Type
Polyclonal Antibody
Species
Human
Uniprot ID
O43505
Immunogen
Recombinant human Beta-1,4-glucuronyltransferase 1 protein (161-415AA)
Raised In
Rabbit
Species Reactivity
Human
Tested Applications
ELISA;Not yet tested in other applications.
Background / 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 LARGE, during synthesis of phosphorylated O-mannosyl glycan (PubMed:25279699, PubMed:25279697). Phosphorylated O-mannosyl glycan is a carbohydrate is a carbohydrate structure present in alpha-dystroglycan (DAG1), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity (PubMed:25279699, PubMed:25279697). Required for axon guidance; via its function in O-mannosylation of alpha-dystroglycan (DAG1).
Isotype
IgG
Conjugate
Biotin
Storage Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form
Liquid
Storage
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze.
Purity
Caprylic Acid Ammonium Sulfate Precipitation purified
Literature
[1]”Tumor suppressor function of laminin-binding alpha-dystroglycan requires a distinct beta3-N-acetylglucosaminyltransferase.”Bao X., Kobayashi M., Hatakeyama S., Angata K., Gullberg D., Nakayama J., Fukuda M.N., Fukuda M.Proc. Natl. Acad. Sci. U.S.A. 106:12109-12114(2009). [2]”Missense mutations in beta-1,3-N-acetylglucosaminyltransferase 1 (B3GNT1) cause Walker-Warburg syndrome.”Buysse K., Riemersma M., Powell G., van Reeuwijk J., Chitayat D., Roscioli T., Kamsteeg E.J., van den Elzen C., van Beusekom E., Blaser S., Babul-Hirji R., Halliday W., Wright G.J., Stemple D.L., Lin Y.Y., Lefeber D.J., van Bokhoven H.Hum. Mol. Genet. 22:1746-1754(2013). [3]”The glucuronyltransferase B4GAT1 is required for initiation of LARGE-mediated alpha-dystroglycan functional glycosylation.”Willer T., Inamori K.I., Venzke D., Harvey C., Morgensen G., Hara Y., Beltran Valero de Bernabe D., Yu L., Wright K.M., Campbell K.P.Elife 3:0-0(2014).Additional information
| Size | 50μg, 100μg |
|---|
