B4GALT7 Antibody (PACO52874)
- SKU:
- PACO52874
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- WB
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | B4GALT7 Antibody (PACO52874) |
| Antibody SKU: | PACO52874 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, WB |
| Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:200-1:2000 |
| Species Reactivity: | Human, Mouse |
| Immunogen: | Recombinant Human Beta-1,4-galactosyltransferase 7 protein (52-327AA) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | Western Blot. Positive WB detected in: Mouse brain tissue. All lanes: B4GALT7 antibody at 3µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 38 kDa. Observed band size: 38 kDa. |
| Background: | Required for the biosynthesis of the tetrasaccharide linkage region of proteoglycans, especially for small proteoglycans in skin fibroblasts. |
| Synonyms: | 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)], B4GALT7, XGALT1 |
| UniProt Protein Function: | B4GALT7: Required for the biosynthesis of the tetrasaccharide linkage region of proteoglycans, especially for small proteoglycans in skin fibroblasts. Defects in B4GALT7 are the cause of Ehlers-Danlos syndrome progeroid type (EDSP). 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. Belongs to the glycosyltransferase 7 family. |
| UniProt Protein Details: | Protein type:Glycan Metabolism - chondroitin sulfate biosynthesis; Membrane protein, integral; Glycan Metabolism - heparan sulfate biosynthesis; Transferase; EC 2.4.1.133 Chromosomal Location of Human Ortholog: 5q35.2-q35.3 Cellular Component: Golgi membrane; Golgi apparatus; integral to membrane Molecular Function:beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase activity; manganese ion binding; xylosylprotein 4-beta-galactosyltransferase activity; galactosyltransferase activity Biological Process: chondroitin sulfate metabolic process; glycosaminoglycan biosynthetic process; proteoglycan metabolic process; glycosaminoglycan metabolic process; negative regulation of fibroblast proliferation; carbohydrate metabolic process; fibril organization and biogenesis; protein amino acid N-linked glycosylation; protein modification process; pathogenesis Disease: Ehlers-danlos Syndrome, Progeroid Type, 1 |
| NCBI Summary: | 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. Mutations in this gene have been associated with the progeroid form of Ehlers-Danlos syndrome. [provided by RefSeq, Oct 2009] |
| UniProt Code: | Q9UBV7 |
| NCBI GenInfo Identifier: | 13123990 |
| NCBI Gene ID: | 11285 |
| NCBI Accession: | Q9UBV7.1 |
| UniProt Secondary Accession: | Q9UBV7,Q9UHN2, B3KN39, |
| UniProt Related Accession: | Q9UBV7 |
| Molecular Weight: | 327 |
| NCBI Full Name: | Beta-1,4-galactosyltransferase 7 |
| NCBI Synonym Full Names: | xylosylprotein beta 1,4-galactosyltransferase, polypeptide 7 |
| NCBI Official Symbol: | B4GALT7 |
| NCBI Official Synonym Symbols: | EDSP1; XGPT1; XGALT1 |
| NCBI Protein Information: | beta-1,4-galactosyltransferase 7; beta4Gal-T7; beta4GalT-VII; beta-1,4-GalTase 7; galactosyltransferase I; beta-1,4-galactosyltransferase VII; UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 7; proteoglycan UDP-galactose:beta-xylose beta1,4-galactosyltransferase I; UDP-galactose:beta-N-acetylglucosamine beta-1,4-galactosyltransferase 7 |
| UniProt Protein Name: | Beta-1,4-galactosyltransferase 7 |
| UniProt Synonym Protein Names: | UDP-Gal:beta-GlcNAc beta-1,4-galactosyltransferase 7; UDP-galactose:beta-N-acetylglucosamine beta-1,4-galactosyltransferase 7Including the following 1 domains:Xylosylprotein 4-beta-galactosyltransferase (EC:2.4.1.133)Alternative name(s):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 |
| Protein Family: | Beta-1,4-galactosyltransferase |
| UniProt Gene Name: | B4GALT7 |
| UniProt Entry Name: | B4GT7_HUMAN |
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