Lewis, Caroline and Ohlendieck, Kay (2010) Proteomic profiling of naturally protected extraocular muscles from the dystrophin-deficient mdx mouse. Biochemical and Biophysical Research Communications, 396 (4). pp. 1024-1029. ISSN 0006-291X

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Abstract

Duchenne muscular dystrophy is the most frequent neuromuscular disorder of childhood. Although this x-linked muscle disease is extremely progressive, not all subtypes of skeletal muscles are affected in the same way. While extremities and trunk muscles are drastically weakened, extraocular muscles are usually spared in Duchenne patients. In order to determine the global protein expression pattern in these naturally protected muscles we have performed a comparative proteomic study of the established mdx mouse model of x-linked muscular dystrophy. Fluorescence difference in-gel electrophoretic analysis of 9-week-old dystrophin-deficient versus age-matched normal extraocular muscle, using a pH 4-7 gel range, identified out of 1088 recognized protein spots a moderate expression change in only seven protein species. Desmin, apolipoprotein A-I binding protein and perilipin-3 were found to be increased and gelsolin, gephyrin, transaldolase, and acyl-CoA dehydrogenase were shown to be decreased in mdx extraocular muscles. Immunoblotting revealed a drastic up-regulation of utrophin, comparable levels of beta-dystroglycan and key Ca(2+)-regulatory elements, and an elevated concentration of small stress proteins in mdx extraocular muscles. This suggests that despite the lack of dystrophin only a limited number of cellular systems are perturbed in mdx extraocular muscles, probably due to the substitution of dystrophin by its autosomal homolog. Utrophin appears to prevent the loss of dystrophin-associated proteins and Ca(2+)-handling elements in extraocular muscle tissue. Interestingly, the adaptive mechanisms that cause the sparing of extraocular fibers seem to be closely linked to an enhanced cellular stress response.

Item Type:	Article
Keywords:	Extraocular muscle; MDX; Muscular dystrophy; Muscle proteomics; Utrophin; Heat shock protein;
Academic Unit:	Faculty of Science and Engineering > Biology
Item ID:	7493
Identification Number:	0.1016/j.bbrc.2010.05.052
Depositing User:	Prof. Kay Ohlendieck
Date Deposited:	07 Oct 2016 10:41
Journal or Publication Title:	Biochemical and Biophysical Research Communications
Publisher:	Elsevier
Refereed:	Yes
Funders:	Muscular Dystrophy Ireland, Irish Research Council for Science Engineering and Technology (IRCSET)
URI:	http://www.sciencedirect.com
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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