B4GALT7, Blocking Peptide

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; B4GALT7; XGALT1

For Research Use Only. Not for use in diagnostic procedures.

The synthetic peptide sequence is selected from aa 59-73 of HUMAN B4GALT7

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.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Small volumes of B4GALT7 blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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

37,406 Da

beta-1,4-galactosyltransferase 7

beta-1,4-galactosyltransferase 7

Beta-1,4-galactosyltransferase 7

XGALT1; Beta-1,4-GalTase 7; Beta4Gal-T7; b4Gal-T7  [Similar Products]

B4GT7_HUMAN

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]

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

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