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Animal Bioresource in Japan

.: Home > Animal Bioresource in Japan > 2013 > Volume 62 Number 3 > Nami Masubuchi, Yuichi Shidoh, Shunzo Kondo, Jun Takatoh, Kazunori Hanaoka

Subcellular Localization of Dystrophin Isoforms in Cardiomyocytes and Phenotypic Analysis of Dystrophin-deficient Mice Reveal Cardiac Myopathy is Predominantly Caused by a Deficiency in Full-length Dystrophin

Nami Masubuchi, Yuichi Shidoh, Shunzo Kondo, Jun Takatoh, Kazunori Hanaoka
Laboratory of Molecular Embryology, Department of Bioscience, Kitasato University School of Science, 1–15–1 Kitasato, Minami-Ku, Sagamihara, Kanagawa 252-0373, Japan
Abstract :

Duchenne muscular dystrophy (DMD) is an X-linked recessive progressive muscle degenerative disorder that causes dilated cardiomyopathy in the second decade of life in affected males. Dystrophin, the gene responsible for DMD, encodes full-length dystrophin and various short dystrophin isoforms. In the mouse heart, full-length dystrophin Dp427 and a short dystrophin isoform, Dp71, are expressed. In this study, we intended to clarify the functions of these dystrophin isoforms in DMD-related cardiomyopathy. We used two strains of mice: mdx mice, in which Dp427 was absent but Dp71 was present, and DMD-null mice, in which both were absent. By immunohistochemical staining and density-gradient centrifugation, we found that Dp427 was located in the cardiac sarcolemma and also at the T-tubules, whereas Dp71 was specifically located at the T-tubules. In order to determine whether T tubule-associated Dp71 was involved in DMD-related cardiac disruption, we compared the cardiac phenotypes between DMD-null mice and mdx mice. Both DMD-null mice and mdx mice exhibited severe necrosis, which was followed by fibrosis in cardiac muscle. However, we could not detect a significant difference in myocardial fibrosis between mdx mice and DMD-null mice. Based on the present results, we have shown that cardiac myopathy is caused predominantly by a deficiency of full-length dystrophin Dp427.

Keywords :
cardiomyopathy, dp427, dp71, dystrophin, heart

Date Deposited : 06 Apr 2015 11:54

Last Modified : 06 Apr 2015 11:54

Official URL: http://https://www.jstage.jst.go.jp/browse/expanim/62/1/_contents

Volume 62, Number 3, - 2013

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