ADAMTS13 Antibody - CD BioSciences

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ADAMTS13 Antibody

ADAMTS13 Antibody

SPA-00206

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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 Rabbit
Clonality Polyclonal
Clone No. N/A
Isotype IgG
Immunogen Recombinant Protein corresponding to amino acids: CVGADLQAEMCNTQACEKTQLEFMSQQCARTDGQPLRSSPGGASFYHWGA AVPHSQGDALCRHMCRAIGESFIMKRGDSFLDGTRCMPSGPREDGTLSLC VSGS.
Usage
Application IHC
Dilutions Immunohistochemistry (1:200-1:500)
Reactivity Human
Specificity Specificity of human ADAMTS13 antibody verified on a Protein Array containing target protein plus 383 other non-specific proteins.
Storage & Handling
Storage Buffer PBS (pH 7.2) and 40% Glycerol.
Preservative 0.02% Sodium Azide
Storage Temp. Store at 4 °C for a short term. Aliquot and store at -20 °C for long-term storage.
Handling Avoid freeze-thaw cycles.

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