{"metadata":{"accession":"IPR037578","entry_id":null,"type":"family","go_terms":null,"source_database":"interpro","member_databases":{"pirsf":{"PIRSF002495":"von Willebrand factor"}},"integrated":null,"hierarchy":{"accession":"IPR037578","name":"Von Willebrand factor","type":"Family","children":[]},"name":{"name":"Von Willebrand factor","short":"Von_Willebrand_factor"},"description":[{"text":"<p>Von Willebrand factor (VWF) is a multimeric adhesive protein involved in the initiation and progression of thrombus formation at sites of vascular injury [[cite:PUB00016077]]. 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 [[cite:PUB00087266]].  VWF is also a chaperone for coagulation factor VIII, delivering it to the injury site and protecting it from clearance from the plasma [[cite:PUB00087263]].  The protein is multidomain with four VWFD domains, four TIL domains, three VWFA domains, three VWFC domains and a C-terminal CTCK domain [[cite:PUB00087264]].  VWF is cleaved to release von Willebrand antigen 2 by a furin-like endopeptidase [[cite:PUB00087267]].  Von Willebrand diseases 1, 2 and 3 are deficiencies of VWF, resulting in impaired platelet aggregation and prolonged bleeding after trauma [[cite:PUB00087265]].  Von Willebrand disease 3 results in haemophilia.</p>","llm":false,"checked":false,"updated":false}],"wikipedia":null,"literature":{"PUB00087264":{"PMID":22490677,"ISBN":null,"volume":"120","issue":"2","year":2012,"title":"Sequence and structure relationships within von Willebrand factor.","URL":null,"raw_pages":"449-58","medline_journal":"Blood","ISO_journal":"Blood","authors":["Zhou YF","Eng ET","Zhu J","Lu C","Walz T","Springer TA."],"DOI_URL":"https://doi.org/10.1182/blood-2012-01-405134"},"PUB00087265":{"PMID":27959741,"ISBN":null,"volume":"375","issue":"21","year":2016,"title":"Von Willebrand's Disease.","URL":null,"raw_pages":"2067-2080","medline_journal":"N Engl J Med","ISO_journal":"N. Engl. J. Med.","authors":["Leebeek FW","Eikenboom JC."],"DOI_URL":"https://doi.org/10.1056/NEJMra1601561"},"PUB00087263":{"PMID":10961880,"ISBN":null,"volume":"96","issue":"5","year":2000,"title":"von Willebrand factor storage and multimerization: 2 independent intracellular processes.","URL":null,"raw_pages":"1808-15","medline_journal":"Blood","ISO_journal":"Blood","authors":["Haberichter SL","Fahs SA","Montgomery RR."],"DOI_URL":null},"PUB00016077":{"PMID":12871266,"ISBN":null,"volume":"1","issue":"7","year":2003,"title":"Von Willebrand factor, platelets and endothelial cell interactions.","URL":null,"raw_pages":"1335-42","medline_journal":"J Thromb Haemost","ISO_journal":"J. Thromb. Haemost.","authors":["Ruggeri ZM."],"DOI_URL":"http://dx.doi.org/10.1046/j.1538-7836.2003.00260.x"},"PUB00087266":{"PMID":11943773,"ISBN":null,"volume":"277","issue":"24","year":2002,"title":"Identification of the regulatory elements of the human von Willebrand factor for binding to platelet GPIb. Importance of structural integrity of the regions flanked by the CYS1272-CYS1458 disulfide bond.","URL":null,"raw_pages":"22063-72","medline_journal":"J Biol Chem","ISO_journal":"J. Biol. Chem.","authors":["Nakayama T","Matsushita T","Dong Z","Sadler JE","Jorieux S","Mazurier C","Meyer D","Kojima T","Saito H."],"DOI_URL":"https://doi.org/10.1074/jbc.M201327200"},"PUB00087267":{"PMID":18505722,"ISBN":null,"volume":"283","issue":"30","year":2008,"title":"Substrate cleavage analysis of furin and related proprotein convertases. A comparative study.","URL":null,"raw_pages":"20897-906","medline_journal":"J Biol Chem","ISO_journal":"J. Biol. Chem.","authors":["Remacle AG","Shiryaev SA","Oh ES","Cieplak P","Srinivasan A","Wei G","Liddington RC","Ratnikov BI","Parent A","Desjardins R","Day R","Smith JW","Lebl M","Strongin AY."],"DOI_URL":"https://doi.org/10.1074/jbc.M803762200"}},"set_info":null,"overlaps_with":null,"counters":{"subfamilies":0,"domain_architectures":0,"interactions":0,"matches":236,"pathways":41,"proteins":236,"proteomes":119,"sets":0,"structural_models":{"alphafold":0,"bfvd":0},"structures":0,"taxa":399},"entry_annotations":{},"cross_references":{},"is_llm":false,"is_reviewed_llm":false,"is_updated_llm":false,"representative_structure":null}}