Cardiomyocyte ultrastructural damage in β‐thalassaemic mice

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.
Source: International Journal of Experimental Pathology - Category: Pathology Authors: Tags: Original Article Source Type: research