ADAMTS13 Products

Description

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.

Bioinformatics

Entrez	11093 Human
Uniprot	NP_620594 Human NP_620594.1 Human Q76LX8 Human
Product By Gene ID	11093
Alternate Names	A disintegrin and metalloproteinase with thrombospondin motifs 13 a disintegrin-like and metalloprotease (reprolysin type) with thrombospondintype 1 motif, 13 ADAM metallopeptidase with thrombospondin type 1 motif, 13 ADAM-TS13 ADAMTS-13 DKFZp434C2322 EC 3.4.24.14 EC 3.4.24.82 EC 3.4.24.87 FLJ42993 MGC118899 MGC118900 TTPADAM-TS 13 vWF-cleaving protease vWF-CPC9orf8 VWFCPvon Willebrand factor-cleaving protease

Research Areas for ADAMTS13

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Cancer
Signal Transduction