{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T05:50:19Z","timestamp":1789019419901,"version":"build-2803163510"},"reference-count":80,"publisher":"Elsevier BV","issue":"8","license":[{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T00:00:00Z","timestamp":1754006400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,5,1]],"date-time":"2025-05-01T00:00:00Z","timestamp":1746057600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100010659","name":"Erasmus Universitair Medisch Centrum Rotterdam","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100010659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009425","name":"Landsteiner Foundation for Blood Transfusion Research","doi-asserted-by":"publisher","award":["1707"],"award-info":[{"award-number":["1707"]}],"id":[{"id":"10.13039\/100009425","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003246","name":"Dutch Research Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003246","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008322","name":"CSL Behring","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008322","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008373","name":"Takeda Pharmaceutical Company","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008373","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100022776","name":"Stichting Haemophilia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100022776","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["jthjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Thrombosis and Haemostasis"],"published-print":{"date-parts":[[2025,8]]},"DOI":"10.1016\/j.jtha.2025.04.024","type":"journal-article","created":{"date-parts":[[2025,5,6]],"date-time":"2025-05-06T19:20:42Z","timestamp":1746559242000},"page":"2634-2650","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":6,"title":["von Willebrand disease-specific defects and proteomic signatures in endothelial colony\u2013forming cells"],"prefix":"10.1016","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9206-8712","authenticated-orcid":false,"given":"Sebastiaan N.J.","family":"Laan","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stijn","family":"Groten","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard J.","family":"Dirven","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra E.","family":"B\u00fcrgisser","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank W.G.","family":"Leebeek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iris","family":"van Moort","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maartje","family":"van den Biggelaar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruben","family":"Bierings","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeroen","family":"Eikenboom","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noord","family":"Holland","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martijn","family":"Brands","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sjoerd","family":"Koopman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Bukkems","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Cloesmeijer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Janssen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karin","family":"Fijnvandraat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"Gouw","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ron","family":"Math\u00f4t","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lotte","family":"Haverman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emile","family":"van den Akker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maartje","family":"van den Biggelaar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masja","family":"de Haas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sander","family":"Meijer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Voorberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Del Castillo Alferez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huan","family":"Zhang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johan","family":"Boender","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephan","family":"Meijer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karina","family":"Meijer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean","family":"de Jong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geertje","family":"Goedhart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anske","family":"van der Bom","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mettine","family":"Bos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeroen","family":"Eikenboom","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"van der Meer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastiaan","family":"Laan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saskia","family":"Schols","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zuid","family":"Holland","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruben","family":"Bierings","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lex","family":"Burdorf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marjon","family":"Cnossen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Hazelzet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elise","family":"Huisman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marieke","family":"Kruip","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Leebeek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikki","family":"van Leeuwen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hester","family":"Lingsma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Moniek","family":"de Maat","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iris","family":"van Moort","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suzanne","family":"Polinder","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Reitsma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eliza","family":"Roest","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryanne","family":"Arisz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Romano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wala","family":"Al Arashi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon","family":"van Hoorn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tine","family":"Goedhart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Mussert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diaz","family":"Prameyllawati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carin","family":"Uyl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Jansen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathelijn","family":"Fischer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans Kristian","family":"Ploos van Amstel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf","family":"Urbanus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Minka","family":"Zivkovic","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annelien","family":"Bredenoord","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rieke","family":"van der Graaf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lieke","family":"Baas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roger","family":"Schutgens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mari\u00ebtte","family":"Driessens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jtha.2025.04.024_bib1","doi-asserted-by":"crossref","first-page":"5033","DOI":"10.1182\/blood-2010-09-267492","article-title":"Functional architecture of Weibel\u2013Palade bodies","volume":"117","author":"Valentijn","year":"2011","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib2","doi-asserted-by":"crossref","first-page":"1290","DOI":"10.1016\/j.jtha.2024.01.015","article-title":"A new look at an old body: molecular determinants of Weibel\u2013Palade body composition and von Willebrand factor exocytosis","volume":"22","author":"Hordijk","year":"2024","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib3","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1111\/jth.14322","article-title":"Exocytosis of Weibel\u2013Palade bodies: how to unpack a vascular emergency kit","volume":"17","author":"Schillemans","year":"2019","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib4","doi-asserted-by":"crossref","first-page":"2067","DOI":"10.1056\/NEJMra1601561","article-title":"Von Willebrand\u2019s disease","volume":"375","author":"Leebeek","year":"2016","journal-title":"N Engl J Med"},{"key":"10.1016\/j.jtha.2025.04.024_bib5","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/S0887-7963(96)80086-1","article-title":"von Willebrand disease: pathogenesis, classification, and management","volume":"10","author":"Murray","year":"1996","journal-title":"Transfus Med Rev"},{"key":"10.1016\/j.jtha.2025.04.024_bib6","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1016\/j.thromres.2020.07.051","article-title":"How I treat von Willebrand disease","volume":"196","author":"Castaman","year":"2020","journal-title":"Thromb Res"},{"key":"10.1016\/j.jtha.2025.04.024_bib7","doi-asserted-by":"crossref","first-page":"5317","DOI":"10.1182\/bloodadvances.2021006757","article-title":"Desmopressin response depends on the presence and type of genetic variants in patients with type 1 and type 2 von Willebrand disease","volume":"6","author":"Atiq","year":"2022","journal-title":"Blood Adv"},{"key":"10.1016\/j.jtha.2025.04.024_bib8","doi-asserted-by":"crossref","first-page":"e25","DOI":"10.1111\/hae.13382","article-title":"Acquired Von Willebrand syndrome and response to desmopressin","volume":"24","author":"Biguzzi","year":"2018","journal-title":"Haemophilia"},{"key":"10.1016\/j.jtha.2025.04.024_bib9","doi-asserted-by":"crossref","first-page":"3531","DOI":"10.1182\/blood-2007-08-109231","article-title":"Response to desmopressin is influenced by the genotype and phenotype in type 1 von Willebrand disease (VWD): results from the European Study MCMDM-1VWD","volume":"111","author":"Castaman","year":"2008","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib10","doi-asserted-by":"crossref","first-page":"1824","DOI":"10.1111\/j.1538-7836.2009.03595.x","article-title":"Molecular and phenotypic determinants of the response to desmopressin in adult patients with mild hemophilia A","volume":"7","author":"Castaman","year":"2009","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib11","doi-asserted-by":"crossref","first-page":"2078","DOI":"10.1111\/j.1538-7836.2010.03957.x","article-title":"Blood group significantly influences von Willebrand factor increase and half-life after desmopressin in von Willebrand disease Vicenza","volume":"8","author":"Castaman","year":"2010","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib12","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1055\/s-0038-1653816","article-title":"No influence of blood group on the responsiveness to desmopressin in type I \u201cplatelet normal\u201d von Willebrand\u2019s disease","volume":"73","author":"Castaman","year":"1995","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib13","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1055\/s-0033-1354418","article-title":"Clinical efficacy and determinants of response to treatment with desmopressin in mild hemophilia A","volume":"39","author":"Di Perna","year":"2013","journal-title":"Semin Thromb Hemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib14","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1111\/j.1365-2516.1995.tb00047.x","article-title":"The administration of desmopressin by nasal spray: a dose-determination study in patients with mild haemophilia A or von Willebrand\u2019s disease","volume":"1","author":"Lethagen","year":"1995","journal-title":"Haemophilia"},{"key":"10.1016\/j.jtha.2025.04.024_bib15","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1111\/hae.12173","article-title":"Factor VIII mutation and desmopressin-responsiveness in 62 patients with mild haemophilia A","volume":"19","author":"Nance","year":"2013","journal-title":"Haemophilia"},{"key":"10.1016\/j.jtha.2025.04.024_bib16","doi-asserted-by":"crossref","first-page":"874","DOI":"10.1097\/00043426-200311000-00010","article-title":"Desmopressin (DDAVP) responsiveness in children with von Willebrand disease","volume":"25","author":"Revel\u2013Vilk","year":"2003","journal-title":"J Pediatr Hematol Oncol"},{"key":"10.1016\/j.jtha.2025.04.024_bib17","doi-asserted-by":"crossref","first-page":"947","DOI":"10.1046\/j.1365-2141.2002.03507.x","article-title":"DDAVP challenge tests in boys with mild\/moderate haemophilia A","volume":"117","author":"Revel\u2013Vilk","year":"2002","journal-title":"Br J Haematol"},{"key":"10.1016\/j.jtha.2025.04.024_bib18","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1111\/j.1365-2516.2011.02572.x","article-title":"DDAVP responsiveness in children with mild or moderate haemophilia A correlates with age, endogenous FVIII:C level and with haemophilic genotype","volume":"18","author":"Seary","year":"2012","journal-title":"Haemophilia"},{"key":"10.1016\/j.jtha.2025.04.024_bib19","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1111\/j.1365-2516.2008.01666.x","article-title":"Biologic response to subcutaneous and intranasal therapy with desmopressin in a large Amish kindred with type 2M von Willebrand disease","volume":"14","author":"Sharthkumar","year":"2008","journal-title":"Haemophilia"},{"key":"10.1016\/j.jtha.2025.04.024_bib20","doi-asserted-by":"crossref","first-page":"440","DOI":"10.1160\/TH12-06-0383","article-title":"Response to desmopressin is strongly dependent on F8 gene mutation type in mild and moderate haemophilia A","volume":"109","author":"Stoof","year":"2013","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib21","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/j.thromres.2017.09.025","article-title":"Von Willebrand disease mutation spectrum and associated mutation mechanisms","volume":"159","author":"de Jong","year":"2017","journal-title":"Thromb Res"},{"key":"10.1016\/j.jtha.2025.04.024_bib22","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1182\/blood-2006-05-021105","article-title":"The mutational spectrum of type 1 von Willebrand disease: results from a Canadian cohort study","volume":"109","author":"James","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib23","doi-asserted-by":"crossref","first-page":"2344","DOI":"10.1182\/blood-2017-05-786699","article-title":"Novel insights into the clinical phenotype and pathophysiology underlying low VWF levels","volume":"130","author":"Lavin","year":"2017","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib24","doi-asserted-by":"crossref","DOI":"10.1097\/HS9.0000000000000718","article-title":"Importance of genotyping in von Willebrand disease to elucidate pathogenic mechanisms and variability in phenotype","volume":"6","author":"Atiq","year":"2022","journal-title":"Hemasphere"},{"key":"10.1016\/j.jtha.2025.04.024_bib25","doi-asserted-by":"crossref","first-page":"2375","DOI":"10.1111\/jth.14304","article-title":"Genetic regulation of plasma von Willebrand factor levels in health and disease","volume":"16","author":"Swystun","year":"2018","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib26","doi-asserted-by":"crossref","DOI":"10.1097\/HS9.0000000000000297","article-title":"Von Willebrand disease: from in vivo to in vitro disease models","volume":"3","author":"de Boer","year":"2019","journal-title":"Hemasphere"},{"key":"10.1016\/j.jtha.2025.04.024_bib27","doi-asserted-by":"crossref","first-page":"2762","DOI":"10.1182\/blood-2012-06-434373","article-title":"Analysis of the storage and secretion of von Willebrand factor in blood outgrowth endothelial cells derived from patients with von Willebrand disease","volume":"121","author":"Wang","year":"2013","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib28","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1097\/MBC.0000000000000635","article-title":"Abnormal angiogenesis in blood outgrowth endothelial cells derived from von Willebrand disease patients","volume":"28","author":"Selvam","year":"2017","journal-title":"Blood Coagul Fibrinolysis"},{"key":"10.1016\/j.jtha.2025.04.024_bib29","doi-asserted-by":"crossref","first-page":"2773","DOI":"10.1182\/blood-2012-06-435727","article-title":"Cellular and molecular basis of von Willebrand disease: studies on blood outgrowth endothelial cells","volume":"121","author":"Starke","year":"2013","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib30","doi-asserted-by":"crossref","first-page":"3355","DOI":"10.1016\/j.jtha.2024.08.011","article-title":"Endothelial colony-forming cells in the spotlight: insights into the pathophysiology of von Willebrand disease and rare bleeding disorders","volume":"22","author":"Laan","year":"2024","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib31","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1016\/j.jtha.2024.10.023","article-title":"The severe von Willebrand disease variant p.M771V leads to impaired anterograde trafficking of von Willebrand factor in patient-derived and base-edited endothelial colony-forming cells","volume":"23","author":"B\u00e4r","year":"2025","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib32","doi-asserted-by":"crossref","first-page":"683","DOI":"10.1160\/TH12-04-0244","article-title":"WiN study group. Determinants of bleeding phenotype in adult patients with moderate or severe von Willebrand disease","volume":"108","author":"de Wee","year":"2012","journal-title":"Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib33","doi-asserted-by":"crossref","first-page":"2063","DOI":"10.1111\/j.1538-7836.2010.03975.x","article-title":"ISTH\/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders","volume":"8","author":"Rodeghiero","year":"2010","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib34","doi-asserted-by":"crossref","first-page":"2721","DOI":"10.1111\/jth.14998","article-title":"Endothelial characteristics in healthy endothelial colony forming cells; generating a robust and valid ex vivo model for vascular disease","volume":"18","author":"de Boer","year":"2020","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib35","doi-asserted-by":"crossref","first-page":"1709","DOI":"10.1038\/nprot.2012.093","article-title":"Establishment of outgrowth endothelial cells from peripheral blood","volume":"7","author":"Martin-Ramirez","year":"2012","journal-title":"Nat Protoc"},{"key":"10.1016\/j.jtha.2025.04.024_bib36","doi-asserted-by":"crossref","first-page":"2027","DOI":"10.1016\/j.jtha.2024.03.018","article-title":"Transcriptional and functional profiling identifies inflammation and endothelial-to-mesenchymal transition as potential drivers for phenotypic heterogeneity within a cohort of endothelial colony forming cells","volume":"22","author":"Laan","year":"2024","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib37","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1186\/s12859-021-04344-9","article-title":"CellProfiler 4: improvements in speed, utility and usability","volume":"22","author":"Stirling","year":"2021","journal-title":"BMC Bioinformatics"},{"key":"10.1016\/j.jtha.2025.04.024_bib38","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0278009","article-title":"SYMPHONY consortium. Automated segmentation and quantitative analysis of organelle morphology, localization and content using CellProfiler","volume":"18","author":"Laan","year":"2023","journal-title":"PLOS One"},{"key":"10.1016\/j.jtha.2025.04.024_bib39","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1111\/jth.12460","article-title":"Phosphatidylinositol-3,4,5-triphosphate-dependent Rac exchange factor 1 regulates epinephrine-induced exocytosis of Weibel\u2013Palade bodies","volume":"12","author":"van Hooren","year":"2014","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib40","doi-asserted-by":"crossref","first-page":"1544","DOI":"10.1111\/jth.14558","article-title":"Variability of von Willebrand factor-related parameters in endothelial colony forming cells","volume":"17","author":"de Jong","year":"2019","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib41","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1038\/s42003-024-06649-w","article-title":"The proteomic landscape of in vitro cultured endothelial cells across vascular beds","volume":"7","author":"Groten","year":"2024","journal-title":"Commun Biol"},{"key":"10.1016\/j.jtha.2025.04.024_bib42","doi-asserted-by":"crossref","DOI":"10.1016\/j.mcpro.2022.100279","article-title":"Rapid and in-depth coverage of the (phospho-)proteome with deep libraries and optimal window design for dia-PASEF","volume":"21","author":"Skowronek","year":"2022","journal-title":"Mol Cell Proteomics"},{"key":"10.1016\/j.jtha.2025.04.024_bib43","article-title":"cluster: cluster analysis basics and extensions","author":"Maechler","year":"2023","journal-title":"R package"},{"key":"10.1016\/j.jtha.2025.04.024_bib44","doi-asserted-by":"crossref","first-page":"e47","DOI":"10.1093\/nar\/gkv007","article-title":"limma powers differential expression analyses for RNA-sequencing and microarray studies","volume":"43","author":"Ritchie","year":"2015","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jtha.2025.04.024_bib45","doi-asserted-by":"crossref","first-page":"946","DOI":"10.1214\/16-AOAS920","article-title":"Robust hyperparameter estimation protects against hypervariable genes and improves power to detect differential expression","volume":"10","author":"Phipson","year":"2016","journal-title":"Ann Appl Stat"},{"key":"10.1016\/j.jtha.2025.04.024_bib46","author":"Harrell"},{"key":"10.1016\/j.jtha.2025.04.024_bib47","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1089\/omi.2011.0118","article-title":"clusterProfiler: an R package for comparing biological themes among gene clusters","volume":"16","author":"Yu","year":"2012","journal-title":"Omics"},{"key":"10.1016\/j.jtha.2025.04.024_bib48","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1182\/bloodadvances.2020003265","article-title":"ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease","volume":"5","author":"James","year":"2021","journal-title":"Blood Adv"},{"key":"10.1016\/j.jtha.2025.04.024_bib49","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.1111\/jth.12586","article-title":"von Willebrand disease and aging: an evolving phenotype","volume":"12","author":"Sanders","year":"2014","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib50","doi-asserted-by":"crossref","first-page":"636","DOI":"10.1111\/hae.12664","article-title":"Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease","volume":"21","author":"Rydz","year":"2015","journal-title":"Haemophilia"},{"key":"10.1016\/j.jtha.2025.04.024_bib51","doi-asserted-by":"crossref","first-page":"1414","DOI":"10.1182\/blood.2023022457","article-title":"Type 1 VWD classification revisited: novel insights from combined analysis of the LoVIC and WiN studies","volume":"143","author":"Atiq","year":"2024","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib52","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1182\/blood-2010-01-264507","article-title":"Endothelial von Willebrand factor regulates angiogenesis","volume":"117","author":"Starke","year":"2011","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib53","doi-asserted-by":"crossref","first-page":"1854","DOI":"10.1111\/jth.13112","article-title":"Angiogenic characteristics of blood outgrowth endothelial cells from patients with von Willebrand disease","volume":"13","author":"Groeneveld","year":"2015","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib54","doi-asserted-by":"crossref","first-page":"W782","DOI":"10.1093\/nar\/gkac392","article-title":"WebCSEA: web-based cell-type-specific enrichment analysis of genes","volume":"50","author":"Dai","year":"2022","journal-title":"Nucleic Acids Res"},{"key":"10.1016\/j.jtha.2025.04.024_bib55","doi-asserted-by":"crossref","first-page":"1072","DOI":"10.1038\/ng1642","article-title":"Lymphatic vascular defects promoted by Prox1 haploinsufficiency cause adult-onset obesity","volume":"37","author":"Harvey","year":"2005","journal-title":"Nat Genet"},{"key":"10.1016\/j.jtha.2025.04.024_bib56","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1016\/S0092-8674(00)81511-1","article-title":"Prox1 function is required for the development of the murine lymphatic system","volume":"98","author":"Wigle","year":"1999","journal-title":"Cell"},{"key":"10.1016\/j.jtha.2025.04.024_bib57","doi-asserted-by":"crossref","first-page":"4","DOI":"10.3390\/diagnostics12010004","article-title":"Lymphatic endothelial markers and tumor lymphangiogenesis assessment in human breast cancer","volume":"12","author":"Chen","year":"2021","journal-title":"Diagnostics (Basel)"},{"key":"10.1016\/j.jtha.2025.04.024_bib58","first-page":"4780","article-title":"A complete Rab screening reveals novel insights in Weibel-Palade body exocytosis","volume":"125","author":"Zografou","year":"2012","journal-title":"J Cell Sci"},{"key":"10.1016\/j.jtha.2025.04.024_bib59","doi-asserted-by":"crossref","first-page":"2757","DOI":"10.1182\/blood-2012-05-429936","article-title":"The interplay between the Rab27A effectors Slp4-a and MyRIP controls hormone-evoked Weibel\u2013Palade body exocytosis","volume":"120","author":"Bierings","year":"2012","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib60","doi-asserted-by":"crossref","first-page":"2982","DOI":"10.1038\/sj.emboj.7600319","article-title":"Rab3D and annexin A2 play a role in regulated secretion of vWF, but not tPA, from endothelial cells","volume":"23","author":"Knop","year":"2004","journal-title":"EMBO J"},{"key":"10.1016\/j.jtha.2025.04.024_bib61","doi-asserted-by":"crossref","first-page":"2925","DOI":"10.1021\/pr300010r","article-title":"Proteomic screen identifies IGFBP7 as a novel component of endothelial cell-specific Weibel\u2013Palade bodies","volume":"11","author":"van Breevoort","year":"2012","journal-title":"J Proteome Res"},{"key":"10.1016\/j.jtha.2025.04.024_bib62","doi-asserted-by":"crossref","first-page":"R326","DOI":"10.1016\/S0960-9822(03)00239-2","article-title":"Mechanisms of protein import into mitochondria","volume":"13","author":"Truscott","year":"2003","journal-title":"Curr Biol"},{"key":"10.1016\/j.jtha.2025.04.024_bib63","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1161\/ATVBAHA.123.319939","article-title":"Physical contacts between mitochondria and WPBs participate in WPB maturation","volume":"44","author":"Ma","year":"2024","journal-title":"Arterioscler Thromb Vasc Biol"},{"key":"10.1016\/j.jtha.2025.04.024_bib64","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.71526","article-title":"V-ATPase V0a1 promotes Weibel-Palade body biogenesis through the regulation of membrane fission","volume":"10","author":"Yamazaki","year":"2021","journal-title":"Elife"},{"key":"10.1016\/j.jtha.2025.04.024_bib65","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0270299","article-title":"Acidification of endothelial Weibel-Palade bodies is mediated by the vacuolar-type H+-ATPase","volume":"17","author":"Terglane","year":"2022","journal-title":"PLOS One"},{"key":"10.1016\/j.jtha.2025.04.024_bib66","doi-asserted-by":"crossref","first-page":"1657","DOI":"10.1002\/stem.1414","article-title":"Ex vivo expansion of human outgrowth endothelial cells leads to IL-8\u2013mediated replicative senescence and impaired vasoreparative function","volume":"31","author":"Medina","year":"2013","journal-title":"Stem Cells"},{"key":"10.1016\/j.jtha.2025.04.024_bib67","doi-asserted-by":"crossref","first-page":"2321","DOI":"10.1161\/01.ATV.0000184762.63888.bd","article-title":"PAR-1 activation on human late endothelial progenitor cells enhances angiogenesis in vitro with upregulation of the SDF-1\/CXCR4 system","volume":"25","author":"Smadja","year":"2005","journal-title":"Arterioscler Thromb Vasc Biol"},{"key":"10.1016\/j.jtha.2025.04.024_bib68","doi-asserted-by":"crossref","first-page":"2577","DOI":"10.1182\/blood-2003-08-2770","article-title":"Human endothelial cells derived from circulating progenitors display specific functional properties compared with mature vessel wall endothelial cells","volume":"103","author":"Bompais","year":"2004","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib69","doi-asserted-by":"crossref","DOI":"10.1038\/ncomms14361","article-title":"The molecular basis of endothelial cell plasticity","volume":"8","author":"Dejana","year":"2017","journal-title":"Nat Commun"},{"key":"10.1016\/j.jtha.2025.04.024_bib70","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1038\/nrm.2017.36","article-title":"Vascular heterogeneity and specialization in development and disease","volume":"18","author":"Potente","year":"2017","journal-title":"Nat Rev Mol Cell Biol"},{"key":"10.1016\/j.jtha.2025.04.024_bib71","doi-asserted-by":"crossref","first-page":"1505","DOI":"10.1093\/emboj\/21.7.1505","article-title":"An essential role for Prox1 in the induction of the lymphatic endothelial cell phenotype","volume":"21","author":"Wigle","year":"2002","journal-title":"EMBO J"},{"key":"10.1016\/j.jtha.2025.04.024_bib72","doi-asserted-by":"crossref","first-page":"4223","DOI":"10.1182\/blood-2007-06-097592","article-title":"Lymphatic reprogramming of microvascular endothelial cells by CEA-related cell adhesion molecule-1 via interaction with VEGFR-3 and Prox1","volume":"110","author":"Kilic","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.jtha.2025.04.024_bib73","doi-asserted-by":"crossref","first-page":"1986","DOI":"10.1111\/j.1538-7836.2010.03927.x","article-title":"von Willebrand factor variant p.Arg924Gln marks an allele associated with reduced von Willebrand factor and factor VIII levels","volume":"8","author":"Hickson","year":"2010","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib74","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1186\/s13045-015-0166-9","article-title":"Mutations in the D1 domain of von Willebrand factor impair their propeptide-dependent multimerization, intracellular trafficking and secretion","volume":"8","author":"Yin","year":"2015","journal-title":"J Hematol Oncol"},{"key":"10.1016\/j.jtha.2025.04.024_bib75","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1002\/ajh.20470","article-title":"von Willebrand disease R1374C: type 2A or 2M? A challenge to the revised classification. High frequency in the northwest of Spain (Galicia)","volume":"80","author":"Penas","year":"2005","journal-title":"Am J Hematol"},{"key":"10.1016\/j.jtha.2025.04.024_bib76","doi-asserted-by":"crossref","first-page":"1382","DOI":"10.1161\/CIRCULATIONAHA.109.869156","article-title":"Novel associations of multiple genetic loci with plasma levels of factor VII, factor VIII, and von Willebrand factor: the CHARGE (Cohorts for Heart and Aging Research in Genome Epidemiology) Consortium","volume":"121","author":"Smith","year":"2010","journal-title":"Circulation"},{"key":"10.1016\/j.jtha.2025.04.024_bib77","doi-asserted-by":"crossref","first-page":"956","DOI":"10.1111\/jth.12927","article-title":"CLEC4M and STXBP5 gene variations contribute to von Willebrand factor level variation in von Willebrand disease","volume":"13","author":"Sanders","year":"2015","journal-title":"J Thromb Haemost"},{"key":"10.1016\/j.jtha.2025.04.024_bib78","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1161\/CIRCGENETICS.110.957407","article-title":"Effect of genetic variations in syntaxin-binding protein-5 and syntaxin-2 on von Willebrand factor concentration and cardiovascular risk","volume":"3","author":"van Loon","year":"2010","journal-title":"Circ Cardiovasc Genet"},{"key":"10.1016\/j.jtha.2025.04.024_bib79","doi-asserted-by":"crossref","first-page":"6320","DOI":"10.1038\/s41467-022-33796-7","article-title":"von Willebrand factor links primary hemostasis to innate immunity","volume":"13","author":"Drakeford","year":"2022","journal-title":"Nat Commun"},{"key":"10.1016\/j.jtha.2025.04.024_bib80","doi-asserted-by":"crossref","first-page":"2001","DOI":"10.1111\/jth.15778","article-title":"SYMPHONY consortium: orchestrating personalized treatment for patients with bleeding disorders","volume":"20","author":"Cnossen","year":"2022","journal-title":"J Thromb Haemost"}],"container-title":["Journal of Thrombosis and Haemostasis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1538783625002752?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1538783625002752?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,7,28]],"date-time":"2025-07-28T19:46:22Z","timestamp":1753731982000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1538783625002752"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8]]},"references-count":80,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2025,8]]}},"alternative-id":["S1538783625002752"],"URL":"https:\/\/doi.org\/10.1016\/j.jtha.2025.04.024","relation":{},"ISSN":["1538-7836"],"issn-type":[{"value":"1538-7836","type":"print"}],"subject":[],"published":{"date-parts":[[2025,8]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"von Willebrand disease-specific defects and proteomic signatures in endothelial colony\u2013forming cells","name":"articletitle","label":"Article Title"},{"value":"Journal of Thrombosis and Haemostasis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jtha.2025.04.024","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 The Author(s). Published by Elsevier Inc. on behalf of International Society on Thrombosis and Haemostasis.","name":"copyright","label":"Copyright"}]}}