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1: Arterioscler Thromb Vasc Biol. 2003 Mar 1;23(3):388-96. Epub 2003 Jan 30. Links

Comment in:: Arterioscler Thromb Vasc Biol. 2003 Mar 1;23(3):378.

Platelet activation and the formation of the platelet plug: deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura.

Tsai HM.

Division of Hematology, Montefiore Medical Center and Albert Einstein College of Medicine, 111 E 210th St, Bronx, NY, USA. htsai@montefiore.org

In the circulation, a plasma metalloprotease, ADAMTS13, cleaves von Willebrand factor (vWF) in a shear-dependent manner. This article reviews the role of this cleavage in regulating vWF-platelet interaction and proposes a scheme for understanding how a deficiency of ADAMTS13 results in the development of microthrombi in patients with thrombotic thrombocytopenic purpura.

PMID: 12615692 [PubMed - indexed for MEDLINE]

ReviewRecent advances in thrombotic thrombocytopenic purpura.
Hematology Am Soc Hematol Educ Program. 2004; :407-23.

[Hematology Am Soc Hematol Educ Program. 2004]
ReviewDeficiency of ADAMTS13 in thrombotic thrombocytopenic purpura.
Int J Hematol. 2002 Aug; 76 Suppl 2:132-8.

[Int J Hematol. 2002]
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.

[N Engl J Med. 1998]
ReviewMolecular mechanisms in thrombotic thrombocytopenic purpura.
Semin Thromb Hemost. 2004 Oct; 30(5):549-57.

[Semin Thromb Hemost. 2004]
ReviewAetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease.
Best Pract Res Clin Haematol. 2001 Jun; 14(2):437-54.

[Best Pract Res Clin Haematol. 2001]
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Cited by 3 PubMed Central articles

Shear tango: dance of the ADAMTS13/VWF complex.
de Groot R, Lane DA. Blood. 2008 Sep 1; 112(5):1548-9.

[Blood. 2008]
ReviewCoagulation abnormalities in critically ill patients.
Levi M, Opal SM. Crit Care. 2006; 10(4):222.

[Crit Care. 2006]
Size regulation of von Willebrand factor-mediated platelet thrombi by ADAMTS13 in flowing blood.
Donadelli R, Orje JN, Capoferri C, Remuzzi G, Ruggeri ZM. Blood. 2006 Mar 1; 107(5):1943-50. Epub 2005 Nov 17.

[Blood. 2006]

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Cited by 3 PubMed Central articles

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