Next Article in Journal
Reactive Oxygen Species: Modulators of Phenotypic Switch of Vascular Smooth Muscle Cells
Next Article in Special Issue
The Role of Mitochondrial Calcium Homeostasis in Alzheimer’s and Related Diseases
Previous Article in Journal
Role of Extracellular Vesicles in Epithelial Ovarian Cancer: A Systematic Review
Previous Article in Special Issue
Mitochondrial Ca2+ Dynamics in MCU Knockout C. elegans Worms
Article

The Effect of Deflazacort Treatment on the Functioning of Skeletal Muscle Mitochondria in Duchenne Muscular Dystrophy

1
Department of Biochemistry, Cell Biology and Microbiology, Mari State University, pl. Lenina 1, 424001 Yoshkar-Ola, Russia
2
Laboratory of Mitochondrial Transport, Institute of Theoretical and Experimental Biophysics, Russian Academy of Sciences, Institutskaya 3, 142290 Pushchino, Russia
3
Biophotonics Center, Prokhorov General Physics Institute, Russian Academy of Sciences, Vavilov st. 38, 119991 Moscow, Russia
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2020, 21(22), 8763; https://doi.org/10.3390/ijms21228763
Received: 27 October 2020 / Revised: 17 November 2020 / Accepted: 17 November 2020 / Published: 19 November 2020
(This article belongs to the Special Issue Mitochondrial Calcium Signaling)
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. View Full-Text
Keywords: Duchenne muscular dystrophy; skeletal muscle; deflazacort; mitochondria; Ca2+ uniporter; mitochondrial permeability transition Duchenne muscular dystrophy; skeletal muscle; deflazacort; mitochondria; Ca2+ uniporter; mitochondrial permeability transition
Show Figures

Figure 1

MDPI and ACS Style

Dubinin, M.V.; Talanov, E.Y.; Tenkov, K.S.; Starinets, V.S.; Belosludtseva, N.V.; Belosludtsev, K.N. The Effect of Deflazacort Treatment on the Functioning of Skeletal Muscle Mitochondria in Duchenne Muscular Dystrophy. Int. J. Mol. Sci. 2020, 21, 8763. https://doi.org/10.3390/ijms21228763

AMA Style

Dubinin MV, Talanov EY, Tenkov KS, Starinets VS, Belosludtseva NV, Belosludtsev KN. The Effect of Deflazacort Treatment on the Functioning of Skeletal Muscle Mitochondria in Duchenne Muscular Dystrophy. International Journal of Molecular Sciences. 2020; 21(22):8763. https://doi.org/10.3390/ijms21228763

Chicago/Turabian Style

Dubinin, Mikhail V.; Talanov, Eugeny Y.; Tenkov, Kirill S.; Starinets, Vlada S.; Belosludtseva, Natalia V.; Belosludtsev, Konstantin N. 2020. "The Effect of Deflazacort Treatment on the Functioning of Skeletal Muscle Mitochondria in Duchenne Muscular Dystrophy" Int. J. Mol. Sci. 21, no. 22: 8763. https://doi.org/10.3390/ijms21228763

Find Other Styles
Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Article Access Map by Country/Region

1
Search more from Scilit
 
Search
Back to TopTop