Product Description

B4GT7 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES9450-50, ES9450-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: This gene is a member of the beta-1,4-galactosyltransferase (beta4GalT) family. Family members encode type II membrane-bound glycoproteins that appear to have exclusive specificity for the donor substrate UDP-galactose. Each beta4GalT member 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 which then remains uncleaved to function as a transmembrane anchor. The enzyme encoded by this gene attaches the first galactose in the common carbohydrate-protein linkage (GlcA-beta1,3-Gal-beta1,3-Gal-beta1,4-Xyl-beta1-O-Ser) found in proteoglycans. This enzyme differs from other beta4GalTs because it lacks the conserved Cys residues found in beta4GalT1-beta4GalT6 and it is located in cis-Golgi instead of trans-Golgi. M

Reactivity: Human, Mouse

Host: Rabbit

Applications: WB, ELISA

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from part region of human protein

Isotype: IgG

Target: B4GT7

Specificity: B4GT7 Polyclonal Antibody detects endogenous levels of protein.

Dilution: WB 1:500-2000 ELISA 1:5000-20000

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

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Expression: High expression in heart, pancreas and liver, medium in placenta and kidney, low in brain, skeletal muscle and lung,

Observed Band (KD): 35kD

Fields: Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate, Glycosaminoglycan biosynthesis - heparan sulfate / heparin, Metabolic pathways

Function: Catalytic Activity: UDP-galactose + O-beta-D-xylosylprotein = UDP + 4-beta-D-galactosyl-O-beta-D-xylosylprotein
Cofactor: Manganese
Disease: Defects in B4GALT7 are the cause of progeroid Ehlers-Danlos syndrome (EDS) [MIM: 130070]. EDSP is a variant form of Ehlers-Danlos syndrome characterized by progeroid facies, mild mental retardation, short stature, skin hyperextensibility, moderate skin fragility, joint hypermobility principally in digits
Function: Required for the biosynthesis of the tetrasaccharide linkage region of proteoglycans, especially for small proteoglycans in skin fibroblasts
Online Information: Beta-1,4-galactosyltransferase 7,Online Information: GlycoGene database,Pathway: Protein modification, protein glycosylation
Similarity: Belongs to the glycosyltransferase 7 family
Subcellular Location: Cis cisternae of Golgi stack
Tissue Specificity: High expression in heart, pancreas

Subcellular Location: Golgi apparatus, Golgi stack membrane, Single-pass type II membrane protein Cis cisternae of Golgi stack

Storage and Stability: -20°C/1 year

Gene Name: B4GALT7 XGALT1 UNQ748/PRO1478

Protein Name: Beta-1,4-galactosyltransferase 7 (Beta-1,4-GalTase 7) (Beta4Gal-T7) (b4Gal-T7) (EC 2.4.1.-) (UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 7) (UDP-galactose:beta-N-acetylglucosamine beta-1,4-gala

Mouse SwissProt Number: Q8R087

Research Use Only