{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T13:05:35Z","timestamp":1792674335598,"version":"4.5.0"},"reference-count":52,"publisher":"Elsevier BV","issue":"14","license":[{"start":{"date-parts":[[2017,7,1]],"date-time":"2017-07-01T00:00:00Z","timestamp":1498867200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Molecular Biology"],"published-print":{"date-parts":[[2017,7]]},"DOI":"10.1016\/j.jmb.2017.05.013","type":"journal-article","created":{"date-parts":[[2017,5,20]],"date-time":"2017-05-20T08:18:18Z","timestamp":1495268298000},"page":"2161-2177","source":"Crossref","is-referenced-by-count":27,"title":["Enhanced Local Disorder in a Clinically Elusive von Willebrand Factor Provokes High-Affinity Platelet Clumping"],"prefix":"10.1016","volume":"429","author":[{"given":"Alexander","family":"Tischer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Venkata R.","family":"Machha","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan P.","family":"Frontroth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria A.","family":"Brehm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tobias","family":"Obser","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reinhard","family":"Schneppenheim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leland","family":"Mayne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Walter Englander","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew","family":"Auton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jmb.2017.05.013_bb0005","doi-asserted-by":"crossref","first-page":"1412","DOI":"10.1182\/blood-2014-05-378638","article-title":"Von Willebrand factor: Jedi knight of the bloodstream","volume":"124","author":"Springer","year":"2014","journal-title":"Blood"},{"key":"10.1016\/j.jmb.2017.05.013_bb0010","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1111\/j.1365-2516.2007.01643.x","article-title":"Von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) expert panel report (USA)","volume":"14","author":"Nichols","year":"2008","journal-title":"Haemophilia"},{"key":"10.1016\/j.jmb.2017.05.013_bb0015","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1111\/ijlh.12345","article-title":"Laboratory diagnosis of von Willebrand disease","volume":"37","author":"Roberts","year":"2015","journal-title":"Int. J. Lab. Hematol."},{"key":"10.1016\/j.jmb.2017.05.013_bb0020","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1053\/beha.2001.0134","article-title":"A molecular approach to the classification of von Willebrand disease","volume":"14","author":"Schneppenheim","year":"2001","journal-title":"Best Pract. Res. Clin. Haematol."},{"key":"10.1016\/j.jmb.2017.05.013_bb0025","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1111\/jth.12221","article-title":"Structure\u2013function and regulation of ADAMTS-13 protease","volume":"11","author":"Zheng","year":"2013","journal-title":"J. Thromb. Haemost."},{"key":"10.1016\/j.jmb.2017.05.013_bb0035","doi-asserted-by":"crossref","first-page":"9046","DOI":"10.1074\/jbc.271.15.9046","article-title":"Conformational changes in the A1 domain of von Willebrand factor modulating the interaction with platelet glycoprotein Ibalpha","volume":"271","author":"Miyata","year":"1996","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0040","doi-asserted-by":"crossref","first-page":"6586","DOI":"10.1074\/jbc.274.10.6586","article-title":"Distinct structural attributes regulating von Willebrand factor A1 domain interaction with platelet glycoprotein Ibalpha under flow","volume":"274","author":"Miyata","year":"1999","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0045","doi-asserted-by":"crossref","first-page":"1192","DOI":"10.1016\/j.bpj.2010.06.002","article-title":"The mechanism of VWF-mediated platelet GPIb\u03b1 binding","volume":"99","author":"Auton","year":"2010","journal-title":"Biophys. J."},{"key":"10.1016\/j.jmb.2017.05.013_bb0050","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1002\/prot.24464","article-title":"A molten globule intermediate of the von Willebrand factor A1 domain firmly tethers platelets under shear flow","volume":"82","author":"Tischer","year":"2014","journal-title":"Proteins"},{"key":"10.1016\/j.jmb.2017.05.013_bb0055","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1055\/s-0038-1650599","article-title":"Characterization of recombinant von Willebrand factors mutated on cysteine 509 or 695","volume":"76","author":"Siguret","year":"1996","journal-title":"Thromb. Haemost."},{"key":"10.1016\/j.jmb.2017.05.013_bb0060","doi-asserted-by":"crossref","first-page":"2821","DOI":"10.1016\/S0021-9258(18)53847-3","article-title":"Disulfide bond requirements for assembly of the platelet glycoprotein Ib-binding domain of von Willebrand factor","volume":"268","author":"Azuma","year":"1993","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0065","doi-asserted-by":"crossref","first-page":"12,185","DOI":"10.1016\/S0021-9258(19)50324-6","article-title":"Analysis of structure-function relationships in the platelet membrane glycoprotein Ib-binding domain of von Willebrand's factor by expression of deletion mutants","volume":"268","author":"Sugimoto","year":"1993","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0070","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1111\/j.1365-2141.1992.tb04595.x","article-title":"Defects in type IIA von Willebrand disease: a cysteine 509 to arginine substitution in the mature von Willebrand factor disrupts a disulphide loop involved in the interaction with platelet glycoprotein Ib-IX","volume":"82","author":"Lavergne","year":"1992","journal-title":"Br. J. Haematol."},{"key":"10.1016\/j.jmb.2017.05.013_bb0075","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1002\/ajh.10455","article-title":"C1272S: a new candidate mutation in type 2A von Willebrand disease that disrupts the disulfide loop responsible for the interaction of VWF with platelet GP Ib-IX","volume":"75","author":"Penas","year":"2004","journal-title":"Am. J. Hematol."},{"key":"10.1016\/j.jmb.2017.05.013_bb0080","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1111\/j.1365-2516.2011.02569.x","article-title":"C1272F: a novel type 2A von Willebrand's disease mutation in A1 domain; its clinical significance","volume":"18","author":"Woods","year":"2012","journal-title":"Haemophilia"},{"key":"10.1016\/j.jmb.2017.05.013_bb0085","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1111\/j.1365-2516.2007.01610.x","article-title":"The use of desmopressin in von Willebrand disease: the experience of the first 30 years (1977\u20132007)","volume":"14","author":"Federici","year":"2008","journal-title":"Haemophilia"},{"key":"10.1016\/j.jmb.2017.05.013_bb0090","series-title":"PO644-MON, ISTH 2015 Congress Meeting, Toronto, Canada","article-title":"Thrombocytopenia during DDAVP test in two children with type 2A von Willebrand Disease","author":"Frontroth","year":"2015"},{"key":"10.1016\/j.jmb.2017.05.013_bb0095","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1038\/79639","article-title":"Von Willebrand factor conformation and adhesive function is modulated by an internalized water molecule","volume":"7","author":"Celikel","year":"2000","journal-title":"Nat. Struct. Biol."},{"key":"10.1016\/j.jmb.2017.05.013_bb0100","doi-asserted-by":"crossref","first-page":"1176","DOI":"10.1126\/science.107355","article-title":"Structures of glycoprotein Ib\u03b1 and its complex with von Willebrand factor A1 domain","volume":"297","author":"Huizinga","year":"2002","journal-title":"Science"},{"key":"10.1016\/j.jmb.2017.05.013_bb0105","doi-asserted-by":"crossref","first-page":"23,327","DOI":"10.1074\/jbc.M401659200","article-title":"Crystal structure of the wild-type von Willebrand factor A1-glycoprotein Ib\u03b1 complex reveals conformation differences with a complex bearing von Willebrand disease mutations","volume":"279","author":"Dumas","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0110","doi-asserted-by":"crossref","first-page":"5565","DOI":"10.1074\/jbc.M113.511220","article-title":"Structural basis of regulation of von Willebrand factor binding to glycoprotein Ib","volume":"289","author":"Blenner","year":"2014","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0115","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1016\/j.bpj.2014.07.026","article-title":"Misfolding of VWF to pathologically disordered conformations impacts the severity of von Willebrand disease","volume":"107","author":"Tischer","year":"2014","journal-title":"Biophys. J."},{"key":"10.1016\/j.jmb.2017.05.013_bb0120","doi-asserted-by":"crossref","first-page":"13,737","DOI":"10.1073\/pnas.0907195106","article-title":"Conformational selection or induced fit: a flux description of reaction mechanism","volume":"106","author":"Hammes","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jmb.2017.05.013_bb0125","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1056\/NEJM198310063091402","article-title":"Platelet aggregation induced by 1-desamino-8-D-arginine vasopressin (DDAVP) in type IIB von Willebrand's disease","volume":"309","author":"Holmberg","year":"1983","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.jmb.2017.05.013_bb0130","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1055\/s-0037-1614447","article-title":"Post-DDAVP thrombocytopenia in type 2B von Willebrand disease is not associated with platelet consumption: failure to demonstrate glycocalicin increase or platelet activation","volume":"81","author":"Casonato","year":"1999","journal-title":"Thromb. Haemost."},{"key":"10.1016\/j.jmb.2017.05.013_bb0135","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1182\/blood-2014-11-613935","article-title":"Variable content of von Willebrand factor mutant monomer drives the phenotypic variability in a family with von Willebrand disease","volume":"126","author":"Chen","year":"2015","journal-title":"Blood"},{"key":"10.1016\/j.jmb.2017.05.013_bb0140","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1093\/protein\/5.3.191","article-title":"Quantitative analysis of protein far UV circular dichroism spectra by neural networks","volume":"5","author":"Bohm","year":"1992","journal-title":"Protein Eng."},{"key":"10.1016\/j.jmb.2017.05.013_bb0145","doi-asserted-by":"crossref","first-page":"349","DOI":"10.2174\/1389203003381315","article-title":"The use of circular dichroism in the investigation of protein structure and function","volume":"1","author":"Kelly","year":"2000","journal-title":"Curr. Protein Pept. Sci."},{"key":"10.1016\/j.jmb.2017.05.013_bb0150","doi-asserted-by":"crossref","first-page":"1627","DOI":"10.1021\/ac60214a047","article-title":"Smoothing and differentiation of data by simplified least squares procedures","volume":"36","author":"Savitzky","year":"1964","journal-title":"Anal. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0155","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1016\/j.bpj.2009.05.009","article-title":"Changes in thermodynamic stability of von Willebrand factor differentially affect the force-dependent binding to platelet GPIb\u03b1","volume":"97","author":"Auton","year":"2009","journal-title":"Biophys. J."},{"key":"10.1016\/j.jmb.2017.05.013_bb0160","doi-asserted-by":"crossref","first-page":"26,658","DOI":"10.1074\/jbc.M113.453803","article-title":"A mechanism for localized dynamics-driven affinity regulation of the binding of von Willebrand factor to platelet glycoprotein Ib\u03b1","volume":"288","author":"Liu","year":"2013","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0165","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1016\/j.bpj.2015.06.008","article-title":"Structural origins of misfolding propensity in the platelet adhesive von Willebrand factor A1 domain","volume":"109","author":"Zimmermann","year":"2015","journal-title":"Biophys. J."},{"key":"10.1016\/j.jmb.2017.05.013_bb0170","doi-asserted-by":"crossref","first-page":"3848","DOI":"10.1074\/jbc.M115.703850","article-title":"Mutational constraints on local unfolding inhibit the rheological adaptation of von Willebrand factor","volume":"291","author":"Tischer","year":"2016","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0175","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1055\/s-0033-1353442","article-title":"Platelet-type von Willebrand disease: new insights into the molecular pathophysiology of a unique platelet defect","volume":"39","author":"Othman","year":"2013","journal-title":"Semin. Thromb. Hemost."},{"key":"10.1016\/j.jmb.2017.05.013_bb0180","doi-asserted-by":"crossref","first-page":"9420","DOI":"10.1073\/pnas.1202604109","article-title":"A thermodynamic definition of protein domains","volume":"109","author":"Porter","year":"2012","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jmb.2017.05.013_bb0185","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1586\/ehm.11.45","article-title":"Platelet-type von Willebrand disease update: the disease, the molecule and the animal model","volume":"4","author":"Othman","year":"2011","journal-title":"Expert. Rev. Hematol."},{"key":"10.1016\/j.jmb.2017.05.013_bb0190","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1160\/TH12-04-0189","article-title":"A novel D235Y mutation in the GP1BA gene enhances platelet interaction with von Willebrand factor in an Iranian family with platelet-type von Willebrand disease","volume":"108","author":"Enayat","year":"2012","journal-title":"Thromb. Haemost."},{"key":"10.1016\/j.jmb.2017.05.013_bb0195","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/0022-2836(82)90515-0","article-title":"A simple method for displaying the hydropathic character of a protein","volume":"157","author":"Kyte","year":"1982","journal-title":"J. Mol. Biol."},{"key":"10.1016\/j.jmb.2017.05.013_bb0200","doi-asserted-by":"crossref","first-page":"4648","DOI":"10.1073\/pnas.0801965105","article-title":"Flow induces loop-to-\u03b2-hairpin transition on the \u03b2-switch of platelet glycoprotein Ib\u03b1","volume":"105","author":"Lou","year":"2008","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jmb.2017.05.013_bb0205","doi-asserted-by":"crossref","first-page":"1182","DOI":"10.1016\/j.bpj.2010.05.035","article-title":"Flow-induced \u03b2-hairpin folding of the glycoprotein Iba \u03b2-switch","volume":"99","author":"Zou","year":"2010","journal-title":"Biophys. J."},{"key":"10.1016\/j.jmb.2017.05.013_bb0210","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.bpc.2015.11.002","article-title":"Simulations of flow induced structural transition of the \u03b2-switch region of glycoprotein Ib\u03b1","volume":"209","author":"Han","year":"2015","journal-title":"Biophys. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0215","doi-asserted-by":"crossref","first-page":"22,831","DOI":"10.1074\/jbc.M110.103358","article-title":"Destabilization of the A1 domain in von Willebrand factor dissociates the A1A2A3 tri-domain and provokes spontaneous binding to glycoprotein Ib and platelet activation under shear stress","volume":"285","author":"Auton","year":"2010","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.jmb.2017.05.013_bb0220","doi-asserted-by":"crossref","first-page":"992","DOI":"10.1038\/nature09295","article-title":"A mechanically stabilized receptor-ligand flex-bond important in the vasculature","volume":"466","author":"Kim","year":"2010","journal-title":"Nature"},{"key":"10.1016\/j.jmb.2017.05.013_bb0225","doi-asserted-by":"crossref","first-page":"4648","DOI":"10.1073\/pnas.1501689112","article-title":"Force-induced on-rate switching and modulation by mutations in gain-of-function von Willebrand diseases","volume":"112","author":"Kim","year":"2015","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jmb.2017.05.013_bb0230","doi-asserted-by":"crossref","first-page":"1700","DOI":"10.1016\/j.dib.2016.05.004","article-title":"Data on the purification and crystallization of the loss-of-function von Willebrand disease variant (p.Gly1324Ser) of the von Willebrand factor A1 domain","volume":"7","author":"Campbell","year":"2016","journal-title":"Data Brief"},{"key":"10.1016\/j.jmb.2017.05.013_bb0235","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1002\/pro.2294","article-title":"The linker between the D3 and A1 domains of vWF suppresses A1-GPIb\u03b1 catch bonds by site-specific binding to the A1 domain","volume":"22","author":"Tischer","year":"2013","journal-title":"Protein Sci."},{"key":"10.1016\/j.jmb.2017.05.013_bb0240","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1529\/biophysj.105.066548","article-title":"Catch-bond model derived from allostery explains force-activated bacterial adhesion","volume":"90","author":"Thomas","year":"2006","journal-title":"Biophys. J."},{"key":"10.1016\/j.jmb.2017.05.013_bb0245","doi-asserted-by":"crossref","first-page":"1898","DOI":"10.1007\/s13361-011-0235-4","article-title":"Many overlapping peptides for protein hydrogen exchange experiments by the fragment separation-mass spectrometry method","volume":"22","author":"Mayne","year":"2011","journal-title":"J. Am. Soc. Mass Spectrom."},{"key":"10.1016\/j.jmb.2017.05.013_bb0250","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/bs.mie.2015.06.035","article-title":"Hydrogen exchange mass spectrometry","volume":"566","author":"Mayne","year":"2016","journal-title":"Methods Enzymol."},{"key":"10.1016\/j.jmb.2017.05.013_bb0255","doi-asserted-by":"crossref","first-page":"1906","DOI":"10.1007\/s13361-011-0236-3","article-title":"ExMS: data analysis for HX-MS experiments","volume":"22","author":"Kan","year":"2011","journal-title":"J. Am. Soc. Mass Spectrom."},{"key":"10.1016\/j.jmb.2017.05.013_bb0260","doi-asserted-by":"crossref","first-page":"16,438","DOI":"10.1073\/pnas.1315532110","article-title":"Protein hydrogen exchange at residue resolution by proteolytic fragmentation mass spectrometry analysis","volume":"110","author":"Kan","year":"2013","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.jmb.2017.05.013_bb0265","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1074\/mcp.M100009-MCP200","article-title":"Hydrogen exchange-mass spectrometry: optimization of digestion conditions","volume":"1","author":"Wang","year":"2002","journal-title":"Mol. Cell. Proteomics"}],"container-title":["Journal of Molecular Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022283617302425?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022283617302425?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,7,28]],"date-time":"2022-07-28T19:27:09Z","timestamp":1659036429000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0022283617302425"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,7]]},"references-count":52,"journal-issue":{"issue":"14","published-print":{"date-parts":[[2017,7]]}},"alternative-id":["S0022283617302425"],"URL":"https:\/\/doi.org\/10.1016\/j.jmb.2017.05.013","relation":{},"ISSN":["0022-2836"],"issn-type":[{"value":"0022-2836","type":"print"}],"subject":[],"published":{"date-parts":[[2017,7]]}}}