A disintegrin & metalloproteinase with thrombospondin type 1 motif 13; ADAMTS-13; ADAM-TS 13; ADAM-TS13; von Willebrand factor-cleaving protease; vWF-cleaving protease; vWF-CP (ADAMTS13, C9orf8, UNQ6102/PRO20085)

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Function

Cofactor:

  • binds 1 Zn+2 per subunit
  • binds 4 Ca+2 per subunit

Structure

Compartment

secreted, plasma

Expression

expressed primarily in liver

Pathology

Polymorphism

influence plasmatic ADAMTS13 activity

Pharmacology

Laboratory

More general terms

More specific terms

References

  1. Furlan M et al von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. 1998 Nov 26;339(22):1578-84. PMID: https://www.ncbi.nlm.nih.gov/pubmed/9828245
  2. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998 Nov 26;339(22):1585-94. PMID: https://www.ncbi.nlm.nih.gov/pubmed/9828246
  3. Furlan M et al Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996 May 15;87(10):4223-34. PMID: https://www.ncbi.nlm.nih.gov/pubmed/8639781
  4. UniProt http://www.uniprot.org/uniprot/Q76LX8.html
  5. Wikipedia; Note: ADAMTS13 entry http://en.wikipedia.org/wiki/ADAMTS13
  6. GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=ADAMTS13
  7. SeattleSNPs http://pga.gs.washington.edu/data/adamts13/

Database