{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T13:07:29Z","timestamp":1792156049115,"version":"4.4.0"},"reference-count":31,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2006,4,24]],"date-time":"2006-04-24T00:00:00Z","timestamp":1145836800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2006,6]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Type 2B von Willebrand disease (VWD) is characterised by an increased affinity of von Willebrand factor (VWF) for its platelet receptor glycoprotein Ib (GPIb). This feature is usually studied <jats:italic>in vitro<\/jats:italic> by a ristocetin\u2010dependent VWF platelet\u2010binding assay, which has some limitations as it requires [e.g. (radio)\u2010labelled anti\u2010VWF antibodies and normal formaldehyde\u2010fixed platelets]. We, here, extended the applicability of an enzyme\u2010linked immunosorbent assay\u2010based method previously described for the measurement of ristocetin co\u2010factor activity that used a recombinant fragment of GPIb (rfGPIb<jats:italic>\u03b1<\/jats:italic>) and horseradish peroxidase\u2010labelled rabbit anti\u2010human VWF antibodies for measuring the captured ristocetin\u2010VWF complexes on the rfGPIb<jats:italic>\u03b1<\/jats:italic>. Thirty\u2010one type 2B VWD patients from 15 families with eight different known mutations were studied. VWF in plasma from 28 of these patients bound better than normal VWF at 0\u00b72\u2003mg\/ml ristocetin, with the ratio, optical density (OD) patient\/OD normal pool plasma, higher than 1\u00b78. For two of the three other patients with no enhanced response of plasma VWF, the platelet lysate VWF showed an enhanced binding capacity; for the last patient, the results in other members of the family are unequivocal. We conclude that, this new method for measurement of plasma or platelet VWF\u2010binding capacity offers great advantages for correct type 2B VWD diagnosis.<\/jats:p>","DOI":"10.1111\/j.1365-2141.2006.06095.x","type":"journal-article","created":{"date-parts":[[2006,4,24]],"date-time":"2006-04-24T08:40:05Z","timestamp":1145868005000},"page":"655-663","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Measurement of von Willebrand factor binding to a recombinant fragment of glycoprotein Ib<i>\u03b1<\/i> in an enzyme\u2010linked immunosorbent assay\u2010based method: performances in patients with type 2B von Willebrand disease"],"prefix":"10.1111","volume":"133","author":[{"given":"Claudine","family":"Caron","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lysiane","family":"Hilbert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"Vanhoorelbeke","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans","family":"Deckmyn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenny","family":"Goudemand","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudine","family":"Mazurier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,5,16]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0049-3848(75)90074-2"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.86.10.3793"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1999.01358.x"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2516.2002.00672.x"},{"key":"e_1_2_6_6_1","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1016\/S0021-9258(17)42483-5","article-title":"Von Willebrand factor. 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