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Morphofunctional evaluation and oxidative stress in the intestine of dystrophic (mdx) mice

Abstract

Duchenne's Muscular Dystrophy (DMD) is the most prevalent muscular dystrophy and is caused by the absence of the protein dystrophin which links actin cytoskeleton to plasma membrane to extracellular matrix through dystrophin-glycoprotein complex. In skeletal muscle, the most affected tissue, a mechanical function to dystrophin is proposed, although suggestions that it may affect signaling transduction mechanisms are also suggested. As much less is known concerning the role of dystrophin in the intestine, we decided to investigate its role in the intestine of mdx mice. In a previous research, we have initially demonstrated a lower level of oxidant markers in the intestine of mdx mice, accompanied by a thinner intestinal muscular layer, which, however, have not impaired its contractiity properties in response to either muscarinic or KCl-induced membrane depolarization, and a higher body weight. In order to further explore the role of this protein in the intestine of mdx mice, we now planned the following studies: a) ileum morphology, by further exploring collagen fibers, nervous plexus, and myocyte ultrastructure; b) intestinal redox status by evaluating protein and lipid oxidation, antioxidant capacity and expression of antioxidant enzymes; c) intestinal reactivity by exploring other signaling transduction pathways, and d) body composition and energetic metabolism. The understanding of the role of the protein dystrophin in the mdx intestine will contribute to clarify the distinct functions this protein might have in smooth muscle as compared to skeletal muscle, and certainly will help to suggest new guidelines to alleviate gastrointestinal symptoms usually described in DMD patients. (AU)

Scientific publications
(References retrieved automatically from Web of Science and SciELO through information on FAPESP grants and their corresponding numbers as mentioned in the publications by the authors)
ALVES, GABRIEL A.; SILVA, LUISA R.; ROSA, ELOI F.; ABOULAFIA, JEANNINE; FREYMUELLER-HAAPALAINEN, EDNA; SOUCCAR, CADEN; NOUAILHETAS, VIVIANE L. A. Intestine of dystrophic mice presents enhanced contractile resistance to stretching despite morphological impairment. AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY, v. 306, n. 3, p. G191-G199, FEB 2014. Web of Science Citations: 4.

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