| ELK.No | ES9450 |
| Product name | B4GT7 rabbit pAb |
| Reactivity | Human;Mouse |
| Applications | WB;ELISA |
| Other name | |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 11285 |
| Human Swiss-Prot | Q9UBV7 |
| Source | Rabbit |
| Isotype | IgG |
| 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 link | |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | |
| Mouse gene link | |
| Mouse Swiss-Prot | Q8R087 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| 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. |
| Clonality | Polyclonal |
| 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 | -20°C/1 year |
| Molecular Weight (Da) | |
| 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. |

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