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The Effect of Deflazacort Treatment on the Functioning of Skeletal Muscle Mitochondria in Duchenne Muscular Dystrophy.
Dubinin, Mikhail V; Talanov, Eugeny Yu; Tenkov, Kirill S; Starinets, Vlada S; Belosludtseva, Natalia V; Belosludtsev, Konstantin N.
Afiliación
  • Dubinin MV; Department of Biochemistry, Cell Biology and Microbiology, Mari State University, pl. Lenina 1, 424001 Yoshkar-Ola, Russia.
  • Talanov EY; Laboratory of Mitochondrial Transport, Institute of Theoretical and Experimental Biophysics, Russian Academy of Sciences, Institutskaya 3, 142290 Pushchino, Russia.
  • Tenkov KS; Department of Biochemistry, Cell Biology and Microbiology, Mari State University, pl. Lenina 1, 424001 Yoshkar-Ola, Russia.
  • Starinets VS; Department of Biochemistry, Cell Biology and Microbiology, Mari State University, pl. Lenina 1, 424001 Yoshkar-Ola, Russia.
  • Belosludtseva NV; Laboratory of Mitochondrial Transport, Institute of Theoretical and Experimental Biophysics, Russian Academy of Sciences, Institutskaya 3, 142290 Pushchino, Russia.
  • Belosludtsev KN; Laboratory of Mitochondrial Transport, Institute of Theoretical and Experimental Biophysics, Russian Academy of Sciences, Institutskaya 3, 142290 Pushchino, Russia.
Int J Mol Sci ; 21(22)2020 Nov 19.
Article en En | MEDLINE | ID: mdl-33228255
Duchenne muscular dystrophy (DMD) is a severe hereditary disease caused by a lack of dystrophin, a protein essential for myocyte integrity. Mitochondrial dysfunction is reportedly responsible for DMD. This study examines the effect of glucocorticoid deflazacort on the functioning of the skeletal-muscle mitochondria of dystrophin-deficient mdx mice and WT animals. Deflazacort administration was found to improve mitochondrial respiration of mdx mice due to an increase in the level of ETC complexes (complexes III and IV and ATP synthase), which may contribute to the normalization of ATP levels in the skeletal muscle of mdx animals. Deflazacort treatment improved the rate of Ca2+ uniport in the skeletal muscle mitochondria of mdx mice, presumably by affecting the subunit composition of the calcium uniporter of organelles. At the same time, deflazacort was found to reduce the resistance of skeletal mitochondria to MPT pore opening, which may be associated with a change in the level of ANT2 and CypD. In this case, deflazacort also affected the mitochondria of WT mice. The paper discusses the mechanisms underlying the effect of deflazacort on the functioning of mitochondria and contributing to the improvement of the muscular function of mdx mice.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Pregnenodionas / Regulación de la Expresión Génica / Músculo Esquelético / Distrofia Muscular de Duchenne / Mitocondrias Musculares Límite: Animals Idioma: En Revista: Int J Mol Sci Año: 2020 Tipo del documento: Article País de afiliación: Rusia Pais de publicación: Suiza
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Pregnenodionas / Regulación de la Expresión Génica / Músculo Esquelético / Distrofia Muscular de Duchenne / Mitocondrias Musculares Límite: Animals Idioma: En Revista: Int J Mol Sci Año: 2020 Tipo del documento: Article País de afiliación: Rusia Pais de publicación: Suiza