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B4GALT7, Polyclonal Antibody

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B4GALT7, Polyclonal Antibody


B4GALT7, Polyclonal Antibody  的詳細介紹
Product Name

B4GALT7, Polyclonal Antibody

Full Product Name

B4GALT7 Antibody, Biotin conjugated

Product Synonym Names
Beta-1, 4-galactosyltransferase 7; Beta-1; 4-GalTase 7; Beta4Gal-T7; b4Gal-T7; 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; UNQ748/PRO1478
Product Gene Name

anti-B4GALT7 antibody

[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
AJ005382 mRNA
3D Structure
ModBase 3D Structure for Q9UBV7
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Purity/Purification
>95%, Protein G purified
Form/Format
Liquid
Immunogen
Recombinant Human Beta-1, 4-galactosyltransferase 7 protein (52-327AA)
Conjugation
Biotin
Preservative
0.03% Proclin 300
Constituents
50% Glycerol, 0.01M PBS, pH 7.4
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-B4GALT7 antibody 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.
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Related Product Information for
anti-B4GALT7 antibody
Required for the biosynthesis of the tetrasaccharide linkage region of proteoglycans, especially for small proteoglycans in skin fibroblasts.
Applications Tested/Suitable for anti-B4GALT7 antibody
ELISA (EIA)
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NCBI/Uniprot data below describe general gene information for B4GALT7. It may not necessarily be applicable to this product.
NCBI GI #
6005952
NCBI GeneID
11285
NCBI Accession #
NP_009186.1 [Other Products]
NCBI GenBank Nucleotide #
NM_007255.2 [Other Products]
UniProt Primary Accession #
Q9UBV7 [Other Products]
UniProt Secondary Accession #
Q9UHN2; B3KN39[Other Products]
UniProt Related Accession #
Q9UBV7[Other Products]
Molecular Weight
37,406 Da
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NCBI Official Full Name
beta-1,4-galactosyltransferase 7
NCBI Official Synonym Full Names
beta-1,4-galactosyltransferase 7
NCBI Official Symbol
B4GALT7??[Similar Products]
NCBI Official Synonym Symbols
EDSP1; XGPT1; EDSSLA; XGALT1
??[Similar Products]
NCBI Protein Information
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??[Similar Products]
UniProt Synonym Gene Names
XGALT1; Beta-1,4-GalTase 7; Beta4Gal-T7; b4Gal-T7??[Similar Products]
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NCBI Summary for B4GALT7
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]
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UniProt Comments for B4GALT7
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: EC 2.4.1.133; Glycan Metabolism - chondroitin sulfate biosynthesis; Glycan Metabolism - heparan sulfate biosynthesis; Membrane protein, integral; Transferase

Chromosomal Location of Human Ortholog: 5q35.3

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
Research Articles on B4GALT7
1. Our findings demonstrate that B4GALT7 is the causative gene for LRS. The identification of a unique homozygous mutation argues in favor of a founder effect. B4GALT7 encodes a galactosyltransferase.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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