The Effect of Deflazacort Treatment on the Functioning of Skeletal Muscle Mitochondria in Duchenne Muscular Dystrophy.
Adenine Nucleotide Translocator 2
/ genetics
Adenosine Triphosphate
/ biosynthesis
Animals
Calcium
/ metabolism
Calcium Channels
/ genetics
Peptidyl-Prolyl Isomerase F
/ genetics
Electron Transport Complex III
/ genetics
Electron Transport Complex IV
/ genetics
Gene Expression Regulation
/ drug effects
Male
Mice
Mice, Inbred C57BL
Mice, Inbred mdx
Mitochondria, Muscle
/ drug effects
Mitochondrial Proton-Translocating ATPases
/ genetics
Muscle, Skeletal
/ drug effects
Muscular Dystrophy, Duchenne
/ drug therapy
Pregnenediones
/ pharmacology
Ca2+ uniporter
Duchenne muscular dystrophy
deflazacort
mitochondria
mitochondrial permeability transition
skeletal muscle
Journal
International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791
Informations de publication
Date de publication:
19 Nov 2020
19 Nov 2020
Historique:
received:
27
10
2020
revised:
17
11
2020
accepted:
17
11
2020
entrez:
24
11
2020
pubmed:
25
11
2020
medline:
5
3
2021
Statut:
epublish
Résumé
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
Identifiants
pubmed: 33228255
pii: ijms21228763
doi: 10.3390/ijms21228763
pmc: PMC7699511
pii:
doi:
Substances chimiques
Adenine Nucleotide Translocator 2
0
Calcium Channels
0
Peptidyl-Prolyl Isomerase F
0
Pregnenediones
0
mitochondrial calcium uniporter
0
Adenosine Triphosphate
8L70Q75FXE
Electron Transport Complex IV
EC 1.9.3.1
Mitochondrial Proton-Translocating ATPases
EC 3.6.3.-
Electron Transport Complex III
EC 7.1.1.8
deflazacort
KR5YZ6AE4B
Calcium
SY7Q814VUP
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Russian Science Foundation
ID : 18-75-00011
Organisme : Russian Science Foundation
ID : 20-75-10006
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