antibody

Rabbit B4GALT1 Antibody

MBS004650

0.1 mL

440 USD

polyclonal

rabbit

nonconjugated

human, mouse

western blot, ELISA, enzyme immunoassay

Antibody

Rabbit B4GALT1 Antibody

[B4GALT1]

[beta-1,4-galactosyltransferase 1; Beta-1,4-galactosyltransferase 1; beta-1,4-galactosyltransferase 1; lactose synthase; beta-1,4-GalTase 1; glycoprotein-4-beta-galactosyltransferase 2; UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 1; UDP-galactose:beta-N-acetylglucosamine beta-1,4-galactosyltransferase 1; UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1; UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 1; UDP-galactose:beta-N-acetylglucosamine beta-1,4-galactosyltransferase 1Processed beta-1,4-galactosyltransferase 1Including the following 4 domains:Lactose synthase A protein (EC:2.4.1.22); N-acetyllactosamine synthase (EC:2.4.1.90)Alternative name(s):Nal synthase]

[B4GALT1]

[B4GALT1; B4GALT1; GT1; GTB; CDG2D; GGTB2; B4GAL-T1; beta4Gal-T1; GGTB2; Beta-1,4-GalTase 1; Beta4Gal-T1; b4Gal-T1]

B4GT1_HUMAN

polyclonal

rabbit

Human, mouse

398

B4GALT1 antibody detects endogenous levels of total B4GALT1protein.

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol

1 mg/ml

Store at -20 degree C/1 year

ELISA (EIA), Western Blot (WB)

WB: 1:500~1:1000 . ELISA: 1:20000

Western Blot

Immunogen: The antiserum was produced against synthesized peptide derived from C-terminal of human B4GALT1.

13929462

NP_001488.2

NM_001497.3

P15291

43,920 Da

Asparagine N-linked Glycosylation Pathway (161013); Disease Pathway (530764); Galactose Metabolism Pathway (82931); Galactose Metabolism Pathway (292); Glycosaminoglycan Biosynthesis - Keratan Sulfate Pathway (82983); Glycosaminoglycan Biosynthesis - Keratan Sulfate Pathway (357); Glycosaminoglycan Metabolism Pathway (645297); Glycosphingolipid Biosynthesis - Lacto And Neolacto Series Pathway (82995); Glycosphingolipid Biosynthesis - Lacto And Neolacto Series Pathway (370); Glycosphingolipid Biosynthesis, Neolacto-series, LacCer = NLc4Cer Pathway (413372)

This gene is one of seven beta-1,4-galactosyltransferase (beta4GalT) genes. They encode type II membrane-bound glycoproteins that appear to have exclusive specificity for the donor substrate UDP-galactose; all transfer galactose in a beta1,4 linkage to similar acceptor sugars: GlcNAc, Glc, and Xyl. Each beta4GalT has a distinct function in the biosynthesis of different glycoconjugates and saccharide structures. As type II membrane proteins, they have an N-terminal hydrophobic signal sequence that directs the protein to the Golgi apparatus and which then remains uncleaved to function as a transmembrane anchor. By sequence similarity, the beta4GalTs form four groups: beta4GalT1 and beta4GalT2, beta4GalT3 and beta4GalT4, beta4GalT5 and beta4GalT6, and beta4GalT7. This gene is unique among the beta4GalT genes because it encodes an enzyme that participates both in glycoconjugate and lactose biosynthesis. For the first activity, the enzyme adds galactose to N-acetylglucosamine residues that are either monosaccharides or the nonreducing ends of glycoprotein carbohydrate chains. The second activity is restricted to lactating mammary tissues where the enzyme forms a heterodimer with alpha-lactalbumin to catalyze UDP-galactose + D-glucose UDP + lactose. The two enzymatic forms result from alternate transcription initiation sites and post-translational processing. Two transcripts, which differ only at the 5' end, with approximate lengths of 4.1 kb and 3.9 kb encode the same protein. The longer transcript encodes the type II membrane-bound, trans-Golgi resident protein involved in glycoconjugate biosynthesis. The shorter transcript encodes a protein which is cleaved to form the soluble lactose synthase. [provided by RefSeq, Jul 2008]

B4GALT1: The Golgi complex form catalyzes the production of lactose in the lactating mammary gland and could also be responsible for the synthesis of complex-type N-linked oligosaccharides in many glycoproteins as well as the carbohydrate moieties of glycolipids. Defects in B4GALT1 are the cause of congenital disorder of glycosylation type 2D (CDG2D). CDGs are a family of severe inherited diseases caused by a defect in protein N- glycosylation. They are characterized by under-glycosylated serum proteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. Belongs to the glycosyltransferase 7 family. 2 isoforms of the human protein are produced by alternative initiation. Protein type: EC 2.4.1.90; EC 2.4.1.22; Cell adhesion; Membrane protein, integral; Carbohydrate Metabolism - galactose; Glycan Metabolism - glycosphingolipid biosynthesis - lacto and neolacto series; Cell development/differentiation; Motility/polarity/chemotaxis; EC 2.4.1.38; Glycan Metabolism - keratan sulfate biosynthesis; Glycan Metabolism - N-glycan biosynthesis; Cell surface; Transferase. Chromosomal Location of Human Ortholog: 9p13. Cellular Component: glycocalyx; Golgi apparatus; desmosome; extracellular space; basolateral plasma membrane; brush border membrane; Golgi trans cisterna; integral to membrane; Golgi membrane; membrane; plasma membrane; external side of plasma membrane; filopodium. Molecular Function: beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase activity; protein homodimerization activity; N-acetyllactosamine synthase activity; manganese ion binding; lactose synthase activity; cytoskeletal protein binding; beta-tubulin binding; UDP-galactosyltransferase activity; galactosyltransferase activity; alpha-tubulin binding. Biological Process: keratan sulfate metabolic process; extracellular matrix organization and biogenesis; glycosaminoglycan metabolic process; positive regulation of epithelial cell proliferation involved in wound healing; oligosaccharide biosynthetic process; positive regulation of apoptosis involved in mammary gland involution; regulation of acrosome reaction; pathogenesis; lactose biosynthetic process; negative regulation of cell proliferation; development of secondary sexual characteristics; mammary gland development; single fertilization; epithelial cell development; penetration of zona pellucida; cell adhesion; Notch signaling pathway; binding of sperm to zona pellucida; protein amino acid N-linked glycosylation; regulation of cell motility; post-translational protein modification; angiogenesis involved in wound healing; multicellular organism reproduction; cellular protein metabolic process; branching morphogenesis of a tube; keratan sulfate biosynthetic process; carbohydrate metabolic process; galactose metabolic process; protein amino acid N-linked glycosylation via asparagine; leukocyte migration; acute inflammatory response. Disease: Congenital Disorder Of Glycosylation, Type Iid

0.1 mL

440 USD

0.2 mL

715