| Primary Identifier | IPR037578 | Type | Family |
| Short Name | Von_Willebrand_factor |
| description | Von Willebrand factor (VWF) is a multimeric adhesive protein involved in the initiation and progression of thrombus formation at sites of vascular injury []. VWF allows platelets to adhere to sites of vascular injury, forming a bridge between the sub-endothelial collagen matrix and the platelet-surface receptor complex GPIb-IX-V []. VWF is also a chaperone for coagulation factor VIII, delivering it to the injury site and protecting it from clearance from the plasma []. The protein is multidomain with four VWFD domains, four TIL domains, three VWFA domains, three VWFC domains and a C-terminal CTCK domain []. VWF is cleaved to release von Willebrand antigen 2 by a furin-like endopeptidase []. Von Willebrand diseases 1, 2 and 3 are deficiencies of VWF, resulting in impaired platelet aggregation and prolonged bleeding after trauma []. Von Willebrand disease 3 results in haemophilia. |
