ADAMTS13, TTP and Beyond
نویسنده
چکیده
ADAMTS13 (A Disintegrin And Metalloprotease with ThromboSpondin Type 1 repeats, 13), a plasma metalloprotease, cleaves von Willebrand factor (VWF) [1,2]. This cleavage is crucial for reducing the size of VWF multimers and adhesiveness, thereby preventing excessive platelet aggregation and thrombus formation at sites of vascular injury [3,4]. Deficiency of plasma ADAMTS13 activity could result in an accumulation of newly released ultra large (UL) VWF on endothelial cells where it is synthesized [5] and in blood [6], leading to a potentially fatal syndrome, thrombotic thrombocytopenic purpura (TTP).
منابع مشابه
The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis.
Ultralarge von Willebrand factor (UL-VWF) multimers are thought to play a central role in pathogenesis of the disease thrombotic thrombocytopenic purpura (TTP); however, experimental evidence in support of this hypothesis has been difficult to establish. Therefore, to examine directly the requirement for VWF in TTP pathogenesis, we generated ADAMTS13-deficient mice on a TTP-susceptible genetic ...
متن کاملPathogenicity of Anti-ADAMTS13 Autoantibodies in Acquired Thrombotic Thrombocytopenic Purpura☆
BACKGROUND Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease in which anti-ADAMTS13 autoantibodies cause severe enzyme deficiency. ADAMTS13 deficiency causes the loss of regulation of von Willebrand factor multimeric size and platelet-tethering function, which results in the formation of disseminated microvascular platelet microthrombi. Precisely how anti-ADAMTS13 auto...
متن کاملPotential for Recombinant ADAMTS13 as an Effective Therapy for Acquired Thrombotic Thrombocytopenic Purpura.
OBJECTIVE The metalloprotease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) regulates the size of von Willebrand factor multimers. A deficiency in ADAMTS13 activity is associated with the life-threatening disease thrombotic thrombocytopenic purpura (TTP). The vast majority of patients have acquired TTP, where circulating anti-ADAMTS13 autoantibodie...
متن کاملClinical insights from observations on ADAMTS13 deficiency in liver cirrhosis.
Thromb Haemost 2008; 99: 987–988 Ten years ago ADAMTS13 (A disintegrin and metalloprotease with thrombospondin 1-like repeats) exploded into the awareness of haematologists when a severe deficiency of ADAMTS13 was associated with thrombotic thrombocytopenic purpura (TTP) (1, 2). ADAMTS13 is an enzyme in normal plasma required for proteolysis of von Willebrand factor (VWF) following its secretio...
متن کاملvon Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome.
Whether measurement of ADAMTS13 activity may enable physicians to distinguish thrombotic thrombocytopenic purpura (TTP) from hemolytic uremic syndrome (HUS) is still a controversial issue. Our aim was to clarify whether patients with normal or deficient ADAMTS13 activity could be distinguished in terms of disease manifestations and multimeric patterns of plasma von Willebrand factor (VWF). ADAM...
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عنوان ژورنال:
دوره 2 شماره
صفحات -
تاریخ انتشار 2013