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:	http://jap.physiology.org
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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