Lewis, Caroline and Jockusch, Harald and Ohlendieck, Kay
Proteomic Profiling of the Dystrophin-Deficient
MDX Heart Reveals Drastically Altered Levels of
KeyMetabolic and Contractile Proteins.
Journal of Biomedicine and Biotechnology, 2010 (648501).
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.
||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.
||Proteomic Profiling; Dystrophin-Deficient
KeyMetabolic; Contractile Proteins; Duchenne muscular dystrophy;
||Science & Engineering > Biology
Prof. Kay Ohlendieck
||18 Aug 2011 11:59
|Journal or Publication Title:
||Journal of Biomedicine and Biotechnology
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