Von Willebrand Factor Cleaving Protease (VWF-CP) ELISA Kit for Pigeon

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. [provided by RefSeq, Jul 2013]

ADAMTS13: Cleaves the vWF multimers in plasma into smaller forms. Defects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP); also known as Upshaw-Schulman syndrome (USS). A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; Motility/polarity/chemotaxis; Secreted, signal peptide; Calcium-binding; Protease; EC 3.4.24.87; Extracellular matrix

Chromosomal Location of Human Ortholog: 9q34

Cellular Component: proteinaceous extracellular matrix; extracellular space; cell surface; endoplasmic reticulum lumen

Molecular Function: integrin binding; protein binding; zinc ion binding; metallopeptidase activity; metalloendopeptidase activity; calcium ion binding

Biological Process: integrin-mediated signaling pathway; platelet activation; protein amino acid O-linked glycosylation; cellular protein metabolic process; glycoprotein metabolic process; response to toxin; peptide catabolic process; cell-matrix adhesion; protein processing; post-translational protein modification; proteolysis

Disease: Thrombotic Thrombocytopenic Purpura, Congenital