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    Proteomics reveals drastic increase of extracellular matrix proteins collagen and dermatopontin in the aged mdx diaphragm model of Duchenne muscular dystrophy


    Carberry, Steven and Zweyer, Margit and Swandulla, Dieter and Ohlendieck, Kay (2012) Proteomics reveals drastic increase of extracellular matrix proteins collagen and dermatopontin in the aged mdx diaphragm model of Duchenne muscular dystrophy. International Journal of Molecular Medicine, 30 (2). pp. 229-234. ISSN 1107-3756

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    Abstract

    Duchenne muscular dystrophy is a lethal genetic disease of childhood caused by primary abnormalities in the gene coding for the membrane cytoskeletal protein dystrophin. The mdx mouse is an established animal model of various aspects of X-linked muscular dystrophy and is widely used for studying fundamental mechanisms of dystrophinopathy and testing novel therapeutic approaches to treat one of the most frequent gender-specific diseases in humans. In order to determine global changes in the muscle proteome with the progressive deterioration of mdx tissue with age, we have characterized diaphragm muscle from mdx mice at three ages (8-weeks, 12-months and 22-months) using mass spectrometry-based proteomics. Altered expression levels in diaphragm of 8-week vs. 22-month mice were shown to occur in 11 muscle-associated proteins. Aging in the mdx diaphragm seems to be associated with a drastic increase in the extracellular matrix proteins, collagen and dermatopontin, the molecular chaperone αB-crystallin, and the intermediate filament protein vimentin, suggesting increased accumulation of connective tissue, an enhanced cellular stress response and compensatory stabilization of the weakened membrane cytoskeleton. These proteomic findings establish the aged mdx diaphragm as an excellent model system for studying secondary effects of dystrophin deficiency in skeletal muscle tissue.

    Item Type: Article
    Additional Information: This is an open-access article licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License. The article may be redistributed, reproduced, and reused for non-commercial purposes, provided the original source is properly cited: Carberry, S., Zweyer, M., Swandulla, D., Ohlendieck, K."Proteomics reveals drastic increase of extracellular matrix proteins collagen and dermatopontin in the aged mdx diaphragm model of Duchenne muscular dystrophy". International Journal of Molecular Medicine 30, no. 2 (2012): 229-234. http://dx.doi.org/10.3892/ijmm.2012.1006
    Keywords: collagen; dermatopontin; diaphragm; muscular dystrophy; proteomics;
    Academic Unit: Faculty of Science and Engineering > Biology
    Item ID: 6902
    Identification Number: https://doi.org/10.3892/ijmm.2012.1006
    Depositing User: Prof. Kay Ohlendieck
    Date Deposited: 21 Jan 2016 14:41
    Journal or Publication Title: International Journal of Molecular Medicine
    Publisher: Spandidos Publications
    Refereed: Yes
    Funders: Muscular Dystrophy Ireland, Duchenne Ireland, Hume scholarship, Health Research Board (HRB), Higher Education Authority (HEA)
    URI:

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