Western Blot (WB)
(Anti-B4GALT7 Antibody (N-term)at 1:2000 dilution + mouse skeletal muscle lysatesLysates/proteins at 20 ug per lane.SecondaryGoat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilutionPredicted band size : 37 kDaBlocking/Dilution buffer: 5% NFDM/TBST.)
Rabbit anti-Mouse B4GALT7 Polyclonal Antibody | anti-B4GALT7 antibody
B4GALT7 Antibody (N-term)
Western Blot (WB)
(Anti-B4GALT7 Antibody (N-term)at 1:2000 dilution + mouse skeletal muscle lysatesLysates/proteins at 20 ug per lane.SecondaryGoat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilutionPredicted band size : 37 kDaBlocking/Dilution buffer: 5% NFDM/TBST.)
Western Blot (WB)
(Anti-B4GALT7 Antibody (N-term)at 1:2000 dilution + mouse skeletal muscle lysatesLysates/proteins at 20 ug per lane.SecondaryGoat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilutionPredicted band size : 37 kDaBlocking/Dilution buffer: 5% NFDM/TBST.)
Okajima T.,et al.J. Biol. Chem. 274:22915-22918(1999).
Lo N.-W.,et al.Submitted (APR-1999) to the EMBL/GenBank/DDBJ databases.
Clark H.F.,et al.Genome Res. 13:2265-2270(2003).
Ota T.,et al.Nat. Genet. 36:40-45(2004).
NCBI and Uniprot Product Information
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; Transferase; EC 2.4.1.133; Membrane protein, integral; Glycan Metabolism - heparan sulfate biosynthesis
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; protein amino acid N-linked glycosylation; fibril organization and biogenesis; protein modification process; pathogenesis
Disease: Ehlers-danlos Syndrome, Progeroid Type, 1
Research Articles on B4GALT7
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Product Notes
The B4GALT7 b4galt7 (Catalog #AAA9213455) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The B4GALT7 Antibody (N-term) reacts with Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's B4GALT7 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:2000. Researchers should empirically determine the suitability of the B4GALT7 b4galt7 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "B4GALT7, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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