How autoantibodies cause thrombotic thrombocytopenic purpura
Pathogenicity of Autoantibodies in Acquired TTP
James Crawley (lead researcher)
Imperial College London
Start date: 22 February 2016 (Duration 3 years)
Dr James Crawley and his colleagues at Imperial College London are studying a life-threatening disease called acquired thrombotic thrombocytopenic purpura (TTP) where patients develop blood clots throughout their body.
In healthy people, a blood protein called ADAMTS13 cuts another enzyme called von Willebrand factor into smaller pieces, preventing it from triggering unnecessary blood clotting. Most people with TTP have an autoimmune disease where they develop antibodies against ADAMTS13 that cause severe deficiency of this enzyme. Scientists previously thought that these antibodies prevent ADAMTS13 from working by blocking it. But Dr Crawley has found that the major reason why most patients become deficient in this enzyme is because the antibodies remove ADAMTS13 from the blood. Understanding how this happens and how it leads to disease would reveal new ways to treat it.
In this project, Dr Crawley will investigate exactly how the antibodies in people with TTP bind to the ADAMTS13 protein, and how fast they remove it from the blood. He will also examine how disappearance of this protein from the blood affects a person’s treatment, and how the antibodies in these patients change during treatment.
This research will improve our understanding of TTP and may reveal new ways to both monitor and treat people who have it.
||22 February 2016
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