Target：B4GT7

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

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

Human Gene Id：11285

Human Swiss Prot No：Q9UBV7

Mouse Swiss Prot No：Q8R087

Immunogen：Synthesized peptide derived from part region of human protein

Specificity：B4GT7 Polyclonal Antibody detects endogenous levels of protein.

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

Source：Polyclonal, Rabbit,IgG

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

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Observed Band(KD)：35kD

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

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.

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