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{
"metadata": {
"accession": "IPR037578",
"entry_id": null,
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"pirsf": {
"PIRSF002495": "von Willebrand factor"
}
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"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>",
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"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"
}
},
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"is_llm": false,
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}
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