Carberry, Steven and Zweyer, Margit and Swandulla, Dieter and Ohlendieck, Kay (2012) Profiling of Age-Related Changes in the Tibialis Anterior Muscle Proteome of the mdx Mouse Model of Dystrophinopathy. Journal of Biomedicine and Biotechnology, 2012 (691641). ISSN 1110-7243

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Abstract

X-linked muscular dystrophy is a highly progressive disease of childhood and characterized by primary genetic abnormalities in the dystrophin gene. Senescent mdx specimens were used for a large-scale survey of potential age-related alterations in the dystrophic phenotype, because the established mdx animal model of dystrophinopathy exhibits progressive deterioration of muscle tissue with age. Since the mdx tibialis anteriormuscle is a frequently used model system in muscular dystrophy research, we employed this particular muscle to determine global changes in the dystrophic skeletal muscle proteome. The comparison of mdx mice aged 8 weeks versus 22 months by mass-spectrometry-based proteomics revealed altered expression levels in 8 distinct protein species. Increased levels were shown for carbonic anhydrase, aldolase, and electron transferring flavoprotein, while the expressions of pyruvate kinase, myosin, tropomyosin, and the small heat shock protein Hsp27 were found to be reduced in aged muscle. Immunoblotting confirmed age-dependent changes in the density of key muscle proteins in mdx muscle. Thus, segmental necrosis in mdx tibialis anteriormuscle appears to trigger age-related protein perturbations due to dystrophin deficiency. The identification of novel indicators of progressive muscular dystrophy might be useful for the establishment of a muscle subtype-specific biomarker signature of dystrophinopathy.

Item Type:	Article
Additional Information:	Copyright © 2012 Steven Carberry 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: Steven Carberry, Margit Zweyer, Dieter Swandulla, and Kay Ohlendieck, “Profiling of Age-Related Changes in the Tibialis Anterior Muscle Proteome of the mdx Mouse Model of Dystrophinopathy,” Journal of Biomedicine and Biotechnology, vol. 2012, Article ID 691641, 11 pages, 2012. doi:10.1155/2012/691641
Keywords:	Profiling; Age-Related Changes; Tibialis Anterior; Muscle Proteome; mdx; Mouse Model; Dystrophinopathy; X-linked muscular dystrophy;
Academic Unit:	Faculty of Science and Engineering > Biology
Item ID:	6909
Identification Number:	https://doi.org/10.1155/2012/691641
Depositing User:	Prof. Kay Ohlendieck
Date Deposited:	21 Jan 2016 14:40
Journal or Publication Title:	Journal of Biomedicine and Biotechnology
Publisher:	Hindawi Publishing Corporation
Refereed:	Yes
Funders:	Muscular Dystrophy Ireland, Duchenne Ireland, Hume scholarship, Health Research Board (HRB), Higher Education Authority (HEA)
URI:	http://www.hindawi.com/journals/bmri
Use Licence:	This item is available under a Creative Commons Attribution Non Commercial Share Alike Licence (CC BY-NC-SA). Details of this licence are available here

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