B4GALT7 blocking peptide

B4GALT7 Blocking Peptide (N-term)

• Gene Names: B4GALT7; EDSP1; XGPT1; EDSSLA; XGALT1
• Synonyms: B4GALT7; B4GALT7 Blocking Peptide (N-term); Beta-1;4-galactosyltransferase 7; Beta-1;4-GalTase 7; Beta4Gal-T7; b4Gal-T7; 241-; UDP-Gal:beta-GlcNAc beta-1;4-galactosyltransferase 7; UDP-galactose:beta-N-acetylglucosamine beta-1;4-galactosyltransferase 7; Xylosylprotein 4-beta-galactosyltransferase; 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; XGALT1; B4GALT7 blocking peptide

• Specificity: The synthetic peptide sequence is selected from aa 59-73 of HUMAN B4GALT7
• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 327

• Cellular Location: Golgi apparatus, Golgi stack membrane; Single-pass type II membrane protein. Note: Cis cisternae of Golgi stack
• Tissue Location: High expression in heart, pancreas and liver, medium in placenta and kidney, low in brain, skeletal muscle and lung
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for B4GALT7 blocking peptide

Required for the biosynthesis of the tetrasaccharide linkage region of proteoglycans, especially for small proteoglycans in skin fibroblasts.

NCBI and Uniprot Product Information

• NCBI GI #: 13123990
• NCBI GeneID: 11285
• UniProt Accession #: Q9UBV7; Q9UHN2; B3KN39
• Molecular Weight: 37,406 Da
• NCBI Official Full Name: Beta-1,4-galactosyltransferase 7
• NCBI Official Synonym Full Names: beta-1,4-galactosyltransferase 7
• NCBI Official Symbol: B4GALT7
• NCBI Official Synonym Symbols: EDSP1; XGPT1; EDSSLA; XGALT1
• NCBI Protein Information: beta-1,4-galactosyltransferase 7
• UniProt Protein Name: Beta-1,4-galactosyltransferase 7
• Protein Family: Beta-1,4-galactosyltransferase
• UniProt Gene Name: B4GALT7
• UniProt Synonym Gene Names: XGALT1; Beta-1,4-GalTase 7; Beta4Gal-T7; b4Gal-T7
• UniProt Entry Name: B4GT7_HUMAN

NCBI Description

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 Description

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.

Protein type: Glycan Metabolism - chondroitin sulfate biosynthesis; Glycan Metabolism - heparan sulfate biosynthesis; Transferase; Membrane protein, integral; EC 2.4.1.133

Chromosomal Location of Human Ortholog: 5q35.2-q35.3

Cellular Component: Golgi apparatus; Golgi membrane; integral to membrane

Molecular Function: beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase activity; galactosyltransferase activity; manganese ion binding; protein binding; xylosylprotein 4-beta-galactosyltransferase activity

Biological Process: fibril organization and biogenesis; glycosaminoglycan biosynthetic process; glycosaminoglycan metabolic process; negative regulation of fibroblast proliferation; protein amino acid N-linked glycosylation; protein modification process; proteoglycan metabolic process

Disease: Ehlers-danlos Syndrome, Progeroid Type, 1