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    New pathobiochemical insights into dystrophinopathy from the proteomics of senescent mdx mouse muscle


    Holland, Ashling and Dowling, Paul and Ohlendieck, Kay (2014) New pathobiochemical insights into dystrophinopathy from the proteomics of senescent mdx mouse muscle. Frontiers in Aging Neuroscience, 6 (109). pp. 1-5. ISSN 1663-4365

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    Abstract

    Primary abnormalities in the dystrophin gene cause X-linked muscular dystrophy, a highly progressive muscle wasting disorder of childhood. A spontaneous animal model of Duchenne muscular dystrophy is the mdx mouse, which presents a highly interesting phenotype that exhibits considerable variations in the degree of fiber degeneration in different subtypes of muscles. The idea that aging exacerbates the dystrophic mdx phenotype, as previously indicated by a large number of biochemical and cell biological studies, was clearly confirmed by comparative muscle proteomics. Here we outline recent findings of age-dependent changes in the dystrophin-deficient muscle proteome and contrast these results with the previously established proteomic profile of sarcopenic muscle. Besides comparable perturbations of various biochemical functions, especially striking are similarities in the cellular stress response associated with a drastic up-regulation of small αB-crystallin-like heat shock proteins. Hence, the comparison of large-scale proteomic data sets of natural muscle aging with dystrophic sarcopenia promises to shed light on the differential effect of sarcopenia of old age vs. senescent abnormalities on a mutant dystrophic background.

    Item Type: Article
    Additional Information: © 2014 Holland, Dowling and Ohlendieck. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. This work was supported by project grants from the Irish Higher Education Authority(BioAT programme of PRTLI cycle5)and Muscular Dystrophy Ireland.
    Keywords: aging; dystrophin; dystrophin-glycoproteincomplex; dystrophinopathy; massspectrometry; muscle aging; proteomics; sarcopenia;
    Academic Unit: Faculty of Science and Engineering > Biology
    Item ID: 5634
    Identification Number: https://doi.org/10.3389/fnagi.2014.00109
    Depositing User: Paul Dowling
    Date Deposited: 18 Dec 2014 15:15
    Journal or Publication Title: Frontiers in Aging Neuroscience
    Publisher: Frontiers
    Refereed: Yes
    Funders: Higher Education Authority
    URI:

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