Thrombotic thrombocytopenic purpura: reducing the risk?
von Willebrand factor (vWF) has a key role in initiating platelet aggregation, and thereby thrombus formation, that is dependent on its ability to form appropriately sized multimers. Ultralarge multimers promote the formation of the microvascular thrombi that are hallmarks of the life-threatening condition thrombotic thrombocytopenic purpura (TTP). In this issue of the JCI, Chen et al. show that the drug N-acetylcysteine (NAC) can decrease the size of vWF multimers in vitro and in vivo, resolving thrombi in mice. These data suggest that NAC could potentially be used to treat thrombotic conditions such as TTP.
Biomedical Diagnostics Institute, Dublin City University, and Royal College of Surgeons in Ireland, Dublin, Ireland. michael.berndt@dcu.ie
Michael C
MC
Berndt
Robert K
RK
Andrews
D051722:ADAM Proteins / Q000378:metabolism; D000071120:ADAMTS13 Protein; D000111:Acetylcysteine / Q000494:pharmacology / Q000627:therapeutic use*; D000818:Animals; D006801:Humans; D008562:Membrane Glycoproteins / Q000378:metabolism; D051379:Mice; D010974:Platelet Aggregation / Q000187:drug effects; D019038:Platelet Glycoprotein GPIb-IX Complex; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy*; D013927:Thrombosis / Q000473:pathology; D014841:von Willebrand Factor / Q000737:chemistry / Q000378:metabolism*
D008562:Membrane Glycoproteins; D019038:Platelet Glycoprotein GPIb-IX Complex; C094990:adhesion receptor; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human; D000111:Acetylcysteine
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