Insights into von Willebrand factor proteolysis: clinical implications
| Autor: | Derrick John Bowen, Peter William Collins |
|---|---|
| Rok vydání: | 2006 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities Thrombospondin medicine.medical_specialty Hematology medicine.diagnostic_test biology business.industry Proteolysis Thrombotic thrombocytopenic purpura medicine.disease ADAMTS13 Von Willebrand factor hemic and lymphatic diseases Internal medicine Immunology Von Willebrand disease medicine Disintegrin biology.protein business |
| Zdroj: | British Journal of Haematology. 133:457-467 |
| ISSN: | 1365-2141 0007-1048 |
| Popis: | The proteolysis of von Willebrand factor (VWF) by the recently discovered metalloprotease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin repeats), is a normal processing step in VWF biochemistry. Emerging data indicate that this step may be influenced by a variety of factors, some of which favour increased proteolysis and some of which compromise proteolysis. The former may predispose to bleeding, whilst the latter appears to be the underlying mechanism for thrombotic thrombocytopenic purpura (TTP). The new insights support the concept of "risk" in bleeding, particularly in the case of type 1 von Willebrand disease (VWD), in much the same way that risk is considered in venous thrombosis. This review presents relevant current knowledge of VWF proteolysis by ADAMTS13, and a novel model of how this may be implicated in type 1 VWD is proposed, based on events at the vessel wall at a time of haemostatic challenge. |
| Databáze: | OpenAIRE |
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