von Willebrand factor-cleaving protease Chemical Properties

storage temp. 

-20°C

form 

buffered aqueous solution

biological source

rabbit

Safety Information

WGK Germany 

nwg

Storage Class

10 - Combustible liquids

von Willebrand factor-cleaving protease Usage And Synthesis

Uses

A disintegrin-like and metalloprotease with thrombospondin type 1 motif, 13 (ADAMTS13) also known as von willebrand factor-cleaving protease (VWFCP), has distinct regions like a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 domain and spacer domain rich in cysteine residues. ADAMST13 is highly expressed in the liver and secreted as an active enzyme in the blood. In human chromosome, the gene ADAMTS13 is localised on 9q34.

Biological Activity

A disintegrin-like and metalloprotease with thrombospondin type 13 (ADAMTS13) cleaves von Willebrand factor (VWF) and thereby controls platelet thrombus formation. ADAMTS13 requires zinc, and calcium for its effective catalytic activity. Mutations in ADAMTS13 can lead to life threatening acquired thrombotic thrombocytopenic purpura (TTP) or hemolytic-uremic syndrome. Congenital deficiency of ADAMTS13 leads to Upshaw-Schulman syndrome.