{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,9,24]],"date-time":"2023-09-24T10:02:54Z","timestamp":1695549774247},"reference-count":32,"publisher":"Georg Thieme Verlag KG","issue":"08","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Thromb Haemost"],"published-print":{"date-parts":[[2016,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Treatment of bleeding in patients with Glanzmann\u2019s thrombasthenia (GT) can be hampered by iso-antibodies against the \u03b1IIb\u03b23 integrin, which cause rapid clearance of transfused donor platelets. Type 1 GT patients with a total absence of \u03b1IIb\u03b23 from the platelet surface are known to be susceptible to form such isoantibodies. In this study, we describe a type 1 GT patient with a missense mutation (Gly540Asn) located in the EGF3 domain of the \u03b23 integrin subunit. Cotransfection analysis in CHO cells demonstrates total absence of \u03b1IIb\u03b23 from the surface, based on inappropriate \u03b1IIb maturation. The patient\u2019s serum was reactive with \u03b1IIb\u03b23 and \u03b1v\u03b23 integrins in a capture assay, when platelets and endothelial cells were used. Two specificities could be isolated from the patient\u2019s serum, anti-\u03b1IIb\u03b23 and anti-\u03b1v\u03b23 isoantibodies. Both specificities did not interfere with platelet aggregation. In contrast, isoantibodies against \u03b1v\u03b23, but not against \u03b1IIb\u03b23, were able to disturb endothelial cell adhesion onto vitronectin, triggered endothelial cell apoptosis and interfered with endothelial tube formation. This intriguing finding may explain more recently observed features of fetal\/neonatal iso-immune thrombocytopenia in children from type 1 GT mothers with intracranial haemorrhage, which could be related to anti-endothelial activity of the maternal antibodies. In conclusion, we give evidence that two isoantibody entities exist in type 1 GT patients, which are unequivocally different, both in an immunological and functional sense. Further research on the clinical consequences of immunisation against \u03b1v\u03b23 is required, predominantly in GT patients of childbearing age.<\/jats:p><jats:p>Supplementary Material to this article is available online at www.thrombosis-online.com.<\/jats:p>","DOI":"10.1160\/th15-12-0982","type":"journal-article","created":{"date-parts":[[2016,4,21]],"date-time":"2016-04-21T08:45:16Z","timestamp":1461228316000},"page":"262-271","source":"Crossref","is-referenced-by-count":10,"title":["Immunisation against \u03b1IIb\u03b23 and \u03b1v\u03b23 in a type 1 variant of Glanzmann\u2019s thrombasthenia caused by a missense mutation Gly540Asp on \u03b23"],"prefix":"10.1160","volume":"116","author":[{"given":"Hevi","family":"Wihadmadyatami","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lida","family":"R\u00f6der","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heike","family":"Bergh\u00f6fer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gregor","family":"Bein","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathrin","family":"Heidinger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ulrich J.","family":"Sachs","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sentot","family":"Santoso","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2018,3,9]]},"reference":[{"key":"10.1160\/TH15-12-0982-1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2011-07-365635"},{"key":"10.1160\/TH15-12-0982-2","doi-asserted-by":"publisher","DOI":"10.1055\/s-0032-1306346"},{"key":"10.1160\/TH15-12-0982-3","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02744.x"},{"key":"10.1160\/TH15-12-0982-4","doi-asserted-by":"publisher","DOI":"10.3109\/09537104.2014.998994"},{"key":"10.1160\/TH15-12-0982-5","doi-asserted-by":"publisher","DOI":"10.1016\/j.cca.2009.10.016"},{"key":"10.1160\/TH15-12-0982-6","first-page":"1","volume":"6","author":"Nurden","year":"2006","journal-title":"Orphanet J Rare Dis"},{"key":"10.1160\/TH15-12-0982-7","doi-asserted-by":"publisher","DOI":"10.1053\/j.seminhematol.2005.11.009"},{"key":"10.1160\/TH15-12-0982-8","doi-asserted-by":"publisher","DOI":"10.1007\/s12185-010-0731-5"},{"key":"10.1160\/TH15-12-0982-9","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02230.x"},{"key":"10.1160\/TH15-12-0982-10","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2011.02744.x"},{"key":"10.1160\/TH15-12-0982-11","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejogrb.2005.02.011"},{"key":"10.1160\/TH15-12-0982-12","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2516.2010.02396.x"},{"key":"10.1160\/TH15-12-0982-13","doi-asserted-by":"crossref","first-page":"1562","DOI":"10.1182\/blood.V91.5.1562","volume":"91","author":"Grimaldi","year":"1998","journal-title":"Blood"},{"key":"10.1160\/TH15-12-0982-14","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1182\/blood.V67.2.343.343","volume":"67","author":"Santoso","year":"1986","journal-title":"Blood"},{"key":"10.1160\/TH15-12-0982-15","first-page":"5663","volume":"45","author":"Horton","year":"1985","journal-title":"Cancer Res"},{"key":"10.1160\/TH15-12-0982-16","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0066096"},{"key":"10.1160\/TH15-12-0982-17","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V99.4.1205"},{"key":"10.1160\/TH15-12-0982-18","doi-asserted-by":"publisher","DOI":"10.1111\/j.1537-2995.2009.02438.x"},{"key":"10.1160\/TH15-12-0982-19","doi-asserted-by":"crossref","first-page":"1722","DOI":"10.1182\/blood.V70.6.1722.1722","volume":"70","author":"Kiefel","year":"1987","journal-title":"Blood"},{"key":"10.1160\/TH15-12-0982-20","doi-asserted-by":"publisher","DOI":"10.1093\/molbev\/mst197"},{"key":"10.1160\/TH15-12-0982-21","doi-asserted-by":"publisher","DOI":"10.3181\/00379727-208-43863A"},{"key":"10.1160\/TH15-12-0982-22","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.88.8.3160"},{"key":"10.1160\/TH15-12-0982-23","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1999.01376.x"},{"key":"10.1160\/TH15-12-0982-24","doi-asserted-by":"publisher","DOI":"10.1002\/humu.22776"},{"key":"10.1160\/TH15-12-0982-25","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2141.1998.00824.x"},{"key":"10.1160\/TH15-12-0982-26","doi-asserted-by":"publisher","DOI":"10.1016\/S0049-3848(13)70020-3"},{"key":"10.1160\/TH15-12-0982-27","unstructured":"Boval B, Bellucci S, Boyer-Neumann C, et al. 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