ADAMTS13 Antibody - CD BioSciences

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ADAMTS13 Antibody

ADAMTS13 Antibody

SPA-00209

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Target Information
Target Name ADAMTS13
Gene Abbr. ADAMTS13
Gene ID 11093
Full Name ADAM metallopeptidase with thrombospondin type 1 motif 13
Alias ADAM-TS13, ADAMTS-13, C9orf8, VWFCP, vWF-CP
Introduction ADAMTS13 was first identified by its ability to cleave the ultra large aggregates of vWF into smaller forms in the serum. A thrombocytopenic disorder called thrombic thrombocytopenic purpura (TTR) was shown to be due to a deficiency in the cleavage of von Willebrands factor, and ADAMTS13 was identified as the enzyme responsible. ADAMTS13 cleaves the Tyr1605 to Met1606 bond in the A2 domain of vWF, a process stimulated by shear stress in the circulation and by binding to either platelet glycoprotein IBa or to heparin. The cleaved vWF leads to decreased platelet adhesion. An idiopathic form of TTR is found in patients with auto antibodies to ADAMTS13. The full length ADAMTS-1 sequence codes for a 1,427 amino acid protein, with a predicted mass of 153.6 kDa, but glycosylation and the abundance of cysteine residues gives ADAMTS-13 an apparent molecular weight of about 190 kDa on reduced SDS PAGE gels. The variants reported include 1,371, 1,340 and 344 amino acids, with predicted mass of 130.3, 144.5 and 36.7 kDa respectively. Many bands of varying sizes can be seen on Western blots, between 110-190 kDa, perhaps indicating differentially processed ADAMTS-13.
Product Details
Host Mouse
Clonality Monoclonal
Clone No. 4F12
Isotype IgG2B Kappa
Immunogen ADAMTS13 (NP_620594, 1328 a.a. ~ 1427 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. FINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVLYWESESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT.
Usage
Application ELISA
Reactivity Human
Specificity ADAM metallopeptidase with thrombospondin type 1 motif, 13.
Storage & Handling
Storage Buffer In 1X PBS, pH 7.4.
Preservative No Preservative
Storage Temp. Aliquot and store at -20 °C or -80 °C.
Handling Avoid freeze-thaw cycles.

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