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Proteomic Profiling of the Dystrophin-Deficient MDX Heart Reveals Drastically Altered Levels of KeyMetabolic and Contractile Proteins

Lewis, Caroline and Jockusch, Harald and Ohlendieck, Kay (2010) Proteomic Profiling of the Dystrophin-Deficient MDX Heart Reveals Drastically Altered Levels of KeyMetabolic and Contractile Proteins. Journal of Biomedicine and Biotechnology, 2010 (648501). pp. 1-20. ISSN 1110-7243

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Abstract

Although Duchenne muscular dystrophy is primarily classified as a neuromuscular disease, cardiac complications play an important role in the course of this X-linked inherited disorder. The pathobiochemical steps causing a progressive decline in the dystrophic heart are not well understood. We therefore carried out a fluorescence difference in-gel electrophoretic analysis of 9-month-old dystrophin-deficient versus age-matched normal heart, using the established MDX mouse model of muscular dystrophy-related cardiomyopathy. Out of 2,509 detectable protein spots, 79 2D-spots showed a drastic differential expression pattern, with the concentration of 3 proteins being increased, including nucleoside diphosphate kinase and lamin-A/C, and of 26 protein species being decreased, including ATP synthase, fatty acid binding-protein, isocitrate dehydrogenase, NADH dehydrogenase, porin, peroxiredoxin, adenylate kinase, tropomyosin, actin, and myosin light chains. Hence, the lack of cardiac dystrophin appears to trigger a generally perturbed protein expression pattern in the MDX heart, affecting especially energy metabolism and contractile proteins.

Item Type: Article
Additional Information: Research was supported by a project Grant from Muscular Dystrophy Ireland (MDI-125437), a postgraduate stipend from the Irish Research Council for Science, Engineering and Technology, Fonds der Chemischen Industrie and a Hume scholarship from NUI Maynooth, as well as equipment Grants from the Irish Health Research Board and the Higher Education Authority (HRB-EQ/2003/3, HEARERGS- 07-NUIM, HRB-EQ/2004/2). Copyright © 2010 Caroline Lewis et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Keywords: Proteomic Profiling; Dystrophin-Deficient MDX Heart; KeyMetabolic; Contractile Proteins; Duchenne muscular dystrophy;
Subjects: Science & Engineering > Biology
Item ID: 2649
Identification Number: 10.1155/2010/648501
Depositing User: Prof. Kay Ohlendieck
Date Deposited: 18 Aug 2011 11:59
Journal or Publication Title: Journal of Biomedicine and Biotechnology
Publisher: Hindawi Publishing Corporation
Refereed: Yes
URI:

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