Back
A MOUSE MODEL OF HEREDITARY THROMBOTIC THROMBOCYTOPENIC PURPURA
Abstract number: SYM-W-066
Motto1 D.G.
1Internal Medicine and Pediatrics, University of Iowa, Iowa City, United States
How-to-cite Motto DG. A MOUSE MODEL OF HEREDITARY THROMBOTIC THROMBOCYTOPENIC PURPURA. J Thromb Haemost 2007; 5 Supplement 2: SYM-W-066
Abstract
Thrombotic thrombocytopenic purpura (TTP) is a disorder of blood coagulation classically exhibiting the pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, renal dysfunction and mental status changes. However, the clinical presentation can be quite variable making the diagnosis difficult in many cases. "Hyaline" microthrombi composed primarily of platelets and von Willebrand Factor (VWF) are found in the arterioles of affected organs and represent the pathological hallmark of this disease. Accompanying tissue ischemia is thought to explain the clinical TTP signs and symptoms. Pathogenesis of TTP has been linked to dysfunction of ADAMTS13, a metalloprotease whose only known substrate is VWF. Interestingly, recent observations have demonstrated that ADAMTS13 deficiency likely is necessary, but not sufficient for the development of both familial and acquired TTP. These observations suggest that in addition to ADAMTS13 deficiency, further genetic and/or environmental factors are required for TTP pathogenesis. Recently, a mouse model of TTP was established that recapitulates many of the key clinical features of this disease, including the requirement for further genetic and environmental factors in addition to ADAMTS13 deficiency. Therefore, besides being useful for the direct study of TTP disease pathophysiology in vivo, this mouse model may also prove useful for elucidating some of the important environmental and genetic contributors to disease pathogenesis. In this symposium, recent information gained from the analysis of ADAMTS13-deficient mice will be discussed.
To cite this abstract use the following format:
Journal of Thrombosis and Haemostasis 2007; Volume 5, Supplement 2: abstract number
Session Details
| Date: |
01/08/2007
|
| Time: |
00:00-00:00
|
| Session name: |
XXIst ISTH Congress |
| Subject: |
|
| Location: |
Oxford, UK |
| Presentation type: |
|
| Back to top |
|
