Recombinant Human A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13), Truncated

Uniprot Id

Q76LX8

Target Species

Human

Target Name

ADAMTS13

Target Full Name

A disintegrin and metalloproteinase with thrombospondin motifs 13

Target Function

Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation.

Target Involvement

Thrombotic thrombocytopenic purpura congenital (TTP)

Target Subcellular Location

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

Target Tissue Specificity

Plasma. Expressed primarily in liver.

Target Synonyms

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; ADAM-TS 13; ADAM-TS13; ADAMTS 13; ADAMTS-13; ADAMTS13; ADAMTS13 protein; ATS13_HUMAN; C9orf8; TTP; Von Willebrand factor cleaving protease; von Willebrand factor-cleaving protease; vWF cleaving protease; vWF CP; vWF-cleaving protease; vWF-CP; vWFCP

Target Background

This gene encodes a member of a family of proteins containing several distinct regions, including a metalloproteinase domain, a disintegrin-like domain, and a thrombospondin type 1 (TS) motif. The enzyme encoded by this gene specifically cleaves von Willebrand Factor (vWF). Defects in this gene are associated with thrombotic thrombocytopenic purpura. Alternative splicing results in multiple transcript variants.