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    Proteomic profiling of liver tissue from the mdx-4cv mouse model of Duchenne muscular dystrophy

    Murphy, Sandra and Zweyer, Margit and Henry, Michael and Meleady, Paula and Mundegar, Rustam R. and Swandulla, Dieter and Ohlendieck, Kay (2018) Proteomic profiling of liver tissue from the mdx-4cv mouse model of Duchenne muscular dystrophy. Clinical Proteomics, 15 (34). ISSN 1559-0275

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    Background: Duchenne muscular dystrophy is a highly complex multi-system disease caused by primary abnormalities in the membrane cytoskeletal protein dystrophin. Besides progressive skeletal muscle degeneration, this neuromuscular disorder is also associated with pathophysiological perturbations in many other organs including the liver. To determine potential proteome-wide alterations in liver tissue, we have used a comparative and mass spectrometry-based approach to study the dystrophic mdx-4cv mouse model of dystrophinopathy. Methods: The comparative proteomic profling of mdx-4cv versus wild type liver extracts was carried out with an Orbitrap Fusion Tribrid mass spectrometer. The distribution of identifed liver proteins within protein families and potential protein interaction patterns were analysed by systems bioinformatics. Key fndings on fatty acid binding proteins were confrmed by immunoblot analysis and immunofuorescence microscopy. Results: The proteomic analysis revealed changes in a variety of protein families, afecting especially fatty acid, carbohydrate and amino acid metabolism, biotransformation, the cellular stress response and ion handling in the mdx-4cv liver. Drastically increased protein species were identifed as fatty acid binding protein FABP5, ferritin and calumenin. Decreased liver proteins included phosphoglycerate kinase, apolipoprotein and perilipin. The drastic change in FABP5 was independently verifed by immunoblotting and immunofuorescence microscopy. Conclusions: The proteomic results presented here indicate that the intricate and multifaceted pathogenesis of the mdx-4cv model of dystrophinopathy is associated with secondary alterations in the liver afecting especially fatty acid transportation. Since FABP5 levels were also shown to be elevated in serum from dystrophic mice, this protein might be a useful indicator for monitoring liver changes in X-linked muscular dystrophy.

    Item Type: Article
    Additional Information: © The Author(s) 2018. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Cite as: Murphy, S., Zweyer, M., Henry, M. et al. Proteomic profiling of liver tissue from the mdx-4cv mouse model of Duchenne muscular dystrophy. Clin Proteom 15, 34 (2018).
    Keywords: Dystrophin; Dystrophinopathy; FABP5; Fatty acid binding protein; Fatty acid metabolism; Ferritin; Neuromuscular disease;
    Academic Unit: Faculty of Science and Engineering > Biology
    Item ID: 13143
    Identification Number:
    Depositing User: Prof. Kay Ohlendieck
    Date Deposited: 22 Jul 2020 15:48
    Journal or Publication Title: Clinical Proteomics
    Publisher: BMC
    Refereed: Yes
    Funders: Hume scholarship (Maynooth University), Muscular Dystrophy Ireland, Health Research Board (HRB), Science Foundation Ireland (SFI)
    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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