Rabbit anti-Human B4GALNT1 Polyclonal Antibody | anti-B4GALNT1 antibody

Anti-B4GALNT1 Antibody, Rabbit Polyclonal

Cat. Num. AAA8100812

• Gene Names: B4GALNT1; GALGT; GALNACT; GalNAc-T
• Reactivity: Human
• Applications: Immunohistochemistry
• Purity: Protein A & Antigen Affinity
• Synonyms: B4GALNT1; Polyclonal Antibody; Anti-B4GALNT1 Antibody; Rabbit Polyclonal; B4GALNT1 Antibody; Rabbit PAb; Antigen Affinity Purified; beta-1; 4-N-acetyl-galactosaminyl transferase 1; Anti-GALGT Antibody; Anti-GalNAc-T Antibody; Anti-GALNACT Antibody; Anti-SPG26 Antibody; anti-B4GALNT1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human B4GALNT1
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; 0.2um filtered solution in PBS

• Applicable Applications for anti-B4GALNT1 antibody: Immunohistochemistry-Paraffin (IHC-P)
• Application Notes: IHC-P: 1:500-1:2000
• Immunogen: A synthetic peptide corresponding to the N-terminus of the Human B4GALNT1
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with a synthetic peptide corresponding to the N-terminus of the Human B4GALNT1, and purified by antigen affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Avoid repeated freeze-thaw cycles.

Immunohistochemistry (IHC)

(Immunochemical staining of human B4GALNT1 in human brain with rabbit polyclonal antibody (1:1000, formalin-fixed paraffin embedded sections).)

Related Product Information for anti-B4GALNT1 antibody

BGNT-1.1 in ciliated sensory neuron function and morphogenesis. BGNT-1.1 functions at the trans-Golgi network of sheath cells (glia) to influence dye-filling and cilium length, in a cell non-autonomous manner. Notably, BGNT-1.1 is the orthologue of human B3GNT1/B4GAT1, a glycosyltransferase associated with Walker-Warburg syndrome (WWS). WWS is a multigenic disorder characterised by muscular dystrophy as well as brain and eye anomalies. Dystroglycan is a cell membrane receptor that organizes the basement membrane by binding ligands in the extracellular matrix. Proper glycosylation of the alpha-dystroglycan (alpha-DG) subunit is essential for these activities, and lack thereof results in neuromuscular disease. we had known that FKRP, FKTN, TMEM5 and B4GAT1 (formerly known as B3GNT1) localize to the Golgi and contribute to the O-mannosyl post-phosphorylation modification of alpha-DG. Moreover, we assigned B4GAT1 a function as a xylose beta1, 4-glucuronyltransferase. Nuclear magnetic resonance studies confirmed that a glucuronic acid beta1, 4-xylose disaccharide synthesized by B4GAT1 acts as an acceptor primer that can be elongated by LARGE with the ligand-binding heteropolysaccharide. Recent studies demonstrated that mutations in B3GNT1, an enzyme proposed to be involved in poly-N-acetyllactosamine synthesis, were causal for congenital muscular dystrophy with hypoglycosylation of alpha-dystroglycan (secondary dystroglycanopathies). Since defects in the O-mannosylation protein glycosylation pathway are primarily responsible for dystroglycanopathies and with no established O-mannose initiated structures containing a beta3 linked GlcNAc known, we biochemically interrogated this human enzyme.

NCBI and Uniprot Product Information

• NCBI GI #: 451172101
• NCBI GeneID: 2583
• NCBI Accession #: NP_001263397.1
• NCBI GenBank Nucleotide #: NM_001276468.1
• UniProt Accession #: Q00973
• Molecular Weight: 58,882 Da
• NCBI Official Full Name: beta-1,4 N-acetylgalactosaminyltransferase 1 isoform 2
• NCBI Official Synonym Full Names: beta-1,4-N-acetyl-galactosaminyl transferase 1
• NCBI Official Symbol: B4GALNT1
• NCBI Official Synonym Symbols: GALGT; GALNACT; GalNAc-T
• NCBI Protein Information: beta-1,4 N-acetylgalactosaminyltransferase 1; beta1,4GalNAc-T; beta1-4GalNAc-T; GM2/GD2 synthase; GD2 synthase, GM2 synthase; (N-acetylneuraminyl)-galactosylglucosylceramide; UDP-Gal:betaGlcNAc beta-1,4-N-acetylgalactosaminyltransferase transferase 1; UDP
• UniProt Protein Name: Beta-1,4 N-acetylgalactosaminyltransferase 1
• Protein Family: Beta-1,4 N-acetylgalactosaminyltransferase
• UniProt Gene Name: B4GALNT1
• UniProt Synonym Gene Names: GALGT; SIAT2
• UniProt Entry Name: B4GN1_HUMAN

NCBI Description

GM2 and GD2 gangliosides are sialic acid-containing glycosphingolipids. GalNAc-T is the enzyme involved in the biosynthesis of G(M2) and G(D2) glycosphingolipids. GalNAc-T catalyzes the transfer of GalNAc into G(M3) and G(D3) by a beta-1,4 linkage, resulting in the synthesis of G(M2) and G(D2), respectively. Three transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Feb 2013]

Uniprot Description

B4GALNT1: Involved in the biosynthesis of gangliosides GM2, GD2 and GA2. Belongs to the glycosyltransferase 2 family.

Protein type: EC 2.4.1.92; Membrane protein, integral; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Transferase

Chromosomal Location of Human Ortholog: 12q13.3

Cellular Component: membrane; plasma membrane; integral to Golgi membrane

Molecular Function: (N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase activity

Biological Process: sequestering of lipid; carbohydrate metabolic process; lipid glycosylation; protein amino acid glycosylation; glycosphingolipid metabolic process; spermatogenesis; ganglioside biosynthetic process