This gene encodes a member of the ADAMTS (a disintegrin and metalloproteinase with thrombospondin motif) protein family. Members of the family share several distinct protein modules, including a propeptide region, a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. Individual members of this family differ in the number of C-terminal TS motifs, and some have unique C-terminal domains. The enzyme encoded by this gene is the von Willebrand Factor (vWF)-cleaving protease, which is responsible for cleaving at the site of Tyr842-Met843 of the vWF molecule. A deficiency of this enzyme is associated with thrombotic thrombocytopenic purpura. Alternative splicing of this gene generates multiple transcript variants encoding different isoforms. [provided by RefSeq, Nov 2008].

Cleaves the vWF multimers in plasma into smaller forms. Von Willebrand factor cleaving protease; A disintegrin and metalloproteinase with thrombospondin motifs 13; A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13; A disintegrin like and metalloprotease with thrombospondin type 1 motif 13; ADAM metallopeptidase with thrombospondin type 1 motif 13; ADAM TS 13; ADAM TS13; ADAM-TS 13; ADAM-TS13; ADAMTS 13; ADAMTS-13; ADAMTS13; ADAMTS13 protein; ATS13_HUMAN; C9orf8; TTP; von Willebrand factor-cleaving protease; vWF cleaving protease; vWF CP; vWF-cleaving protease; vWF-CP; vWFCP.

Human

WB,IHC,IF,ICC,FC

WB 1:500-2000
IHC 1:20-200
IF 1:100-1:400
ICC 1:100-1:400
FC 1:100

145kD

11093

Q76LX8

https://www.uniprot.org/uniprotkb/Q76LX8

Secreted. Note=Secretion enhanced by O-fucosylation of TSP type-1 repeats.

Plasma. Expressed primarily in liver.

KLH conjugated synthetic peptide derived from human ADAMTS13

Rabbit

Recombinant

IgG

affinity purified by Protein A

Liquid

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.