Publication: Cardiomyocyte ultrastructural damage in β-thalassaemic mice
Issued Date
2013-10-01
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ISSN
13652613
09599673
09599673
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2-s2.0-84883815018
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Mahidol University
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SCOPUS
Bibliographic Citation
International Journal of Experimental Pathology. Vol.94, No.5 (2013), 336-342
Suggested Citation
Chanita Sanyear, Punnee Butthep, Ramaneeya Nithipongvanich, Pornpan Sirankapracha, Pranee Winichagoon, Suthat Fucharoen, Saovaros Svasti Cardiomyocyte ultrastructural damage in β-thalassaemic mice. International Journal of Experimental Pathology. Vol.94, No.5 (2013), 336-342. doi:10.1111/iep.12044 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/31198
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Title
Cardiomyocyte ultrastructural damage in β-thalassaemic mice
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Abstract
Summary: β-thalassaemia is a hereditary anaemia resulting from the absence or reduction in β-globin chain production. Heart complications related to iron overload are the most serious cause of death in these patients. In this report cardiac pathology of β-thalassaemic mice was evaluated by light and electron microscopy. The study was carried out in thalassaemic mice carrying human β-thalassaemia mutation, IVSII-654 (654), transgenic mice carrying human βE-globin transgene insertion (E4), thalassaemic mice with human βE-globin transgene insertion (654/E4) and homozygous thalassaemic mice rescued by the human βE-globin transgene (R), which is generated by cross-breeding between the 654 and E4 mice. Histology showed iron deposition in cardiac myocytes of 654 and R mice, but the ultrastructural damage was observed only in the R mice when compared with the wild type, 654, E4 and 654/E4 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 R mice cardiomyocytes is consistent with the ultrastructural pathology previously studied in patients with thalassaemia. Thus, this R thalassaemic mouse model is suitable for in vivo pathophysiological study of thalassaemic heart. © 2013 International Journal of Experimental Pathology.