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    Drastic reduction of calsequestrin-like proteins and impaired calcium binding in dystrophic mdx muscle


    Culligan, Kevin and Banville, Niamh and Dowling, Paul and Ohlendieck, Kay (2002) Drastic reduction of calsequestrin-like proteins and impaired calcium binding in dystrophic mdx muscle. Journal of applied physiology, 92 (2). pp. 435-445. ISSN 8750-7587

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    Abstract

    Although the reduction in dystrophin-associated glycoproteins is the primary pathophysiological consequence of the deficiency in dystrophin, little is known about the secondary abnormalities leading to x-linked muscular dystrophy. As abnormal Ca2+ handling may be involved in myonecrosis, we investigated the fate of key Ca2+ regulatory membrane proteins in dystrophic mdx skeletal muscle membranes. Whereas the expression of the ryanodine receptor, the dihydropyridine receptor, the Ca2+-ATPase, and calsequestrin was not affected, a drastic decline in calsequestrin-like proteins of 150–220 kDa was observed in dystrophic microsomes using one-dimensional immunoblotting, two-dimensional immunoblotting with isoelectric focusing, diagonal two-dimensional blotting technique, and immunoprecipitation. In analogy, overall Ca2+ binding was reduced in the sarcoplasmic reticulum of dystrophic muscle. The reduction in Ca2+ binding proteins might be directly involved in triggering impaired Ca2+ sequestration within the lumen of the sarcoplasmic reticulum. Thus disturbed sarcolemmal Ca2+ fluxes seem to influence overall Ca2+homeostasis, resulting in distinct changes in the expression profile of a subset of Ca2+ handling proteins, which might be an important factor in the progressive functional decline of dystrophic muscle fibers.

    Item Type: Article
    Keywords: calcium binding proteins; calcium homeostasis; calcium sequestration; muscular dystrophy; sarcoplasmic reticulum;
    Academic Unit: Faculty of Science and Engineering > Biology
    Item ID: 7342
    Identification Number: https://doi.org/10.1152/japplphysiol.00903.2001
    Depositing User: Paul Dowling
    Date Deposited: 15 Aug 2016 16:05
    Journal or Publication Title: Journal of applied physiology
    Publisher: American Physiological Society
    Refereed: Yes
    Funders: European Commission, Enterprise Ireland (EI), Health Research Board (HRB)
    URI:

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