产品名称
B4GT7 Rabbit Polyclonal Antibody
基因名称
B4GALT7 XGALT1 UNQ748/PRO1478
蛋白名称
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-galactosyltransferase 7) [Includes: Xylosylprotein 4-beta-galactosyltransferase (EC 2.4.1.133) (Proteoglycan UDP-galactose:beta-xylose beta1,4-galactosyltransferase I) (UDP-galactose:beta-xylose beta-1,4-galactosyltransferase) (XGPT) (XGalT-1) (Xylosylprotein beta-1,4-galactosyltransferase)]
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
Q9UBV7
Human Swissprot Link
https://www.uniprot.org/uniprotkb/Q9UBV7/entry
Mouse Swissprot No.
Q8R087
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q8R087
免疫原
Synthesized peptide derived from part region of human protein
特异性
B4GT7 Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
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
组织表达
High expression in heart, pancreas and liver, medium in placenta and kidney, low in brain, skeletal muscle and lung.
细胞定位
Golgi apparatus, Golgi stack membrane; Single-pass type II membrane protein. Cis cisternae of Golgi stack.
信号通路
Chondroitin sulfate biosynthesis;Heparan sulfate biosynthesis;
功能
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 and liver, medium in placenta and kidney, low in brain, skeletal muscle and lung.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
