Please use this identifier to cite or link to this item: http://repositorio.unicamp.br/jspui/handle/REPOSIP/199220
Type: Artigo de periódico
Title: Calcium-binding Proteins In Skeletal Muscles Of The Mdx Mice: Potential Role In The Pathogenesis Of Duchenne Muscular Dystrophy.
Author: Pertille, Adriana
de Carvalho, Candida Luiza Tonizza
Matsumura, Cintia Yuri
Neto, Humberto Santo
Marques, Maria Julia
Abstract: Duchenne muscular dystrophy is one of the most common hereditary diseases. Abnormal ion handling renders dystrophic muscle fibers more susceptible to necrosis and a rise in intracellular calcium is an important initiating event in dystrophic muscle pathogenesis. In the mdx mice, muscles are affected with different intensities and some muscles are spared. We investigated the levels of the calcium-binding proteins calsequestrin and calmodulin in the non-spared axial (sternomastoid and diaphragm), limb (tibialis anterior and soleus), cardiac and in the spared extraocular muscles (EOM) of control and mdx mice. Immunoblotting analysis showed a significant increase of the proteins in the spared mdx EOM and a significant decrease in the most affected diaphragm. Both proteins were comparable to the cardiac muscle controls. In limb and sternomastoid muscles, calmodulin and calsequestrin were affected differently. These results suggest that differential levels of the calcium-handling proteins may be involved in the pathogenesis of myonecrosis in mdx muscles. Understanding the signaling mechanisms involving Ca(2+)-calmodulin activation and calsequestrin expression may be a valuable way to develop new therapeutic approaches to the dystrophinopaties.
Subject: Animals
Blotting, Western
Calmodulin
Calsequestrin
Diaphragm
Disease Models, Animal
Fluorescent Antibody Technique
Male
Mice
Mice, Inbred Mdx
Muscle, Skeletal
Muscular Dystrophy, Duchenne
Myocardium
Necrosis
Phenotype
Rights: fechado
Identifier DOI: 10.1111/j.1365-2613.2009.00688.x
Address: http://www.ncbi.nlm.nih.gov/pubmed/20002835
Date Issue: 2010
Appears in Collections:Artigos e Materiais de Revistas Científicas - Unicamp

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