{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,4]],"date-time":"2023-10-04T05:42:07Z","timestamp":1696398127973},"reference-count":27,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2013,9,10]],"date-time":"2013-09-10T00:00:00Z","timestamp":1378771200000},"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":["Int J Experimental Path"],"published-print":{"date-parts":[[2013,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>\u03b2\u2010thalassaemia is a hereditary anaemia resulting from the absence or reduction in \u03b2\u2010globin chain production. Heart complications related to iron overload are the most serious cause of death in these patients. In this report cardiac pathology of \u03b2\u2010thalassaemic mice was evaluated by light and electron microscopy. The study was carried out in thalassaemic mice carrying human \u03b2\u2010thalassaemia mutation, <jats:styled-content style=\"fixed-case\">IVSII<\/jats:styled-content>\u2010654 (654), transgenic mice carrying human \u03b2<jats:sup>E<\/jats:sup>\u2010globin transgene insertion (E4), thalassaemic mice with human \u03b2<jats:sup>E<\/jats:sup>\u2010globin transgene insertion (654\/E4) and homozygous thalassaemic mice rescued by the human \u03b2<jats:sup>E<\/jats:sup>\u2010globin transgene (R), which is generated by cross\u2010breeding between the 654 and E4 mice. Histology showed iron deposition in cardiac myocytes of 654 and <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content> mice, but the ultrastructural damage was observed only in the <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content> mice when compared with the wild type, 654, <jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>4 and 654\/<jats:styled-content style=\"fixed-case\">E<\/jats:styled-content>4 mice. Histopathological changes in the cardiomyocytes of the R mice included mitochondrial swelling, loss of myofilaments and the presence of lipofuscin, related to the increased level of tissue iron content. The progressive ultrastructural pathology in <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content> mice cardiomyocytes is consistent with the ultrastructural pathology previously studied in patients with thalassaemia. Thus, this <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content> thalassaemic mouse model is suitable for <jats:italic>in vivo<\/jats:italic> pathophysiological study of thalassaemic heart.<\/jats:p>","DOI":"10.1111\/iep.12044","type":"journal-article","created":{"date-parts":[[2013,9,11]],"date-time":"2013-09-11T09:55:45Z","timestamp":1378893345000},"page":"336-342","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Cardiomyocyte ultrastructural damage in \u03b2\u2010thalassaemic mice"],"prefix":"10.1111","volume":"94","author":[{"given":"Chanita","family":"Sanyear","sequence":"first","affiliation":[{"name":"Department of Pathology Faculty of Medicine Ramathibodi Hospital Mahidol University Bangkok Thailand"},{"name":"Thalassemia Research Center Institute of Molecular Biosciences Mahidol University Salaya Campus Phuttamonthon Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Punnee","family":"Butthep","sequence":"additional","affiliation":[{"name":"Department of Pathology Faculty of Medicine Ramathibodi Hospital Mahidol University Bangkok Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ramaneeya","family":"Nithipongvanich","sequence":"additional","affiliation":[{"name":"Thalassemia Research Center Institute of Molecular Biosciences Mahidol University Salaya Campus Phuttamonthon Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pornpan","family":"Sirankapracha","sequence":"additional","affiliation":[{"name":"Thalassemia Research Center Institute of Molecular Biosciences Mahidol University Salaya Campus Phuttamonthon Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pranee","family":"Winichagoon","sequence":"additional","affiliation":[{"name":"Thalassemia Research Center Institute of Molecular Biosciences Mahidol University Salaya Campus Phuttamonthon Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suthat","family":"Fucharoen","sequence":"additional","affiliation":[{"name":"Thalassemia Research Center Institute of Molecular Biosciences Mahidol University Salaya Campus Phuttamonthon Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saovaros","family":"Svasti","sequence":"additional","affiliation":[{"name":"Thalassemia Research Center Institute of Molecular Biosciences Mahidol University Salaya Campus Phuttamonthon Thailand"},{"name":"Department of Biochemistry Faculty of Science Mahidol University Bangkok Thailand"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,9,10]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.10915"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1159\/000205046"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0609.2003.00011.x"},{"key":"e_1_2_8_5_1","first-page":"1187","article-title":"Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine","volume":"89","author":"Borgna\u2010Pignatti C.","year":"2004","journal-title":"Haematologica"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2009.166587"},{"key":"e_1_2_8_7_1","volume-title":"Ultrastructural Pathology of the Cell and Matrix","author":"Ghadially F.N.","year":"1997"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2010.03.083"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2006.08.076"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ygeno.2006.03.009"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-008-9240-1"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1080\/03630260701726491"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.109.913863"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.2010.01549.x"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0609.2012.01779.x"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1080\/01913120050118549"},{"key":"e_1_2_8_17_1","doi-asserted-by":"crossref","first-page":"2152","DOI":"10.1182\/blood.V91.6.2152","article-title":"A common human \u03b2\u2010globin splicing mutation modeled in mice","volume":"91","author":"Lewis J.","year":"1998","journal-title":"Blood"},{"key":"e_1_2_8_18_1","first-page":"147","article-title":"Heart cells in culture: a model of myocardial iron overload and chelation","volume":"106","author":"Link G.","year":"1985","journal-title":"J. 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