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The molecular pathology of this condition represents a range of genetic mechanisms, some of which are now very well characterized, and others that are still under investigation. In general, our knowledge of the molecular basis of type 2 and 3 <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content> is now well advanced, and in some instances this information is being used to enhance clinical management. In contrast, our understanding of the molecular pathogenesis of the most common form of <jats:styled-content style=\"fixed-case\">VWD<\/jats:styled-content>, type 1 disease, is still at an early stage, with preliminary evidence that this phenotype involves a complex interplay between environmental factors and the influence of genetic variability both within and outside of the <jats:italic><jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content><\/jats:italic> locus.<\/jats:p>","DOI":"10.1111\/bjh.12249","type":"journal-article","created":{"date-parts":[[2013,2,14]],"date-time":"2013-02-14T09:58:34Z","timestamp":1360835914000},"page":"166-176","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["The molecular characterization of von <scp>W<\/scp>illebrand disease: good in parts"],"prefix":"10.1111","volume":"161","author":[{"given":"P.D.","family":"James","sequence":"first","affiliation":[{"name":"Department of Medicine Etherington Hall Queen's University Kingston ON Canada"},{"name":"Department of Pathology and Molecular Medicine Richardson Laboratory Queen's University Kingston ON Canada"}]},{"given":"D.","family":"Lillicrap","sequence":"additional","affiliation":[{"name":"Department of Pathology and Molecular Medicine Richardson Laboratory Queen's University Kingston ON Canada"}]}],"member":"311","published-online":{"date-parts":[[2013,2,14]]},"reference":[{"key":"e_1_2_16_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-10-384610"},{"key":"e_1_2_16_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-08-300152"},{"key":"e_1_2_16_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03665.x"},{"key":"e_1_2_16_5_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20120810"},{"key":"e_1_2_16_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-04-1046"},{"key":"e_1_2_16_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-08-109231"},{"key":"e_1_2_16_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2009.03381.x"},{"key":"e_1_2_16_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2012.04661.x"},{"key":"e_1_2_16_10_1","doi-asserted-by":"publisher","DOI":"10.1309\/AJCPH5JK4ONENPAE"},{"key":"e_1_2_16_11_1","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1055\/s-0038-1649589","article-title":"Associations of factor VIII and von Willebrand factor with age, race, sex, and risk factors for atherosclerosis. 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