A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy
We recetly identified a signaling pathway that links the upregulation of miR-379 with a mitochondrial response in dystrophic muscle. In the present commentary, we explain the significance that this pathway may have in mitochondrial dysfunction in Duchenne muscular dystrophy (DMD). We identified the...
Main Authors: | , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
PAGEPress Publications
2021-09-01
|
Series: | European Journal of Translational Myology |
Subjects: | |
Online Access: | https://pagepressjournals.org/index.php/bam/article/view/10012 |
id |
doaj-1926c63cbc56400a9c3850d80aa316b0 |
---|---|
record_format |
Article |
spelling |
doaj-1926c63cbc56400a9c3850d80aa316b02021-09-17T21:20:54ZengPAGEPress PublicationsEuropean Journal of Translational Myology2037-74522037-74602021-09-0110.4081/ejtm.2021.10012A revised model for mitochondrial dysfunction in Duchenne muscular dystrophyAi Vu Hong0Mathilde Sanson1Isabelle Richard2David Israeli3Genethon, Evry, France; Université Paris-Saclay, Univ Evry, Inserm, Généthon, Integrare research unit UMR-S951, EvryGenethon, Evry, France; Université Paris-Saclay, Univ Evry, Inserm, Généthon, Integrare research unit UMR-S951, EvryGenethon, Evry, France; Université Paris-Saclay, Univ Evry, Inserm, Généthon, Integrare research unit UMR-S951, EvryGenethon, Evry, France; Université Paris-Saclay, Univ Evry, Inserm, Généthon, Integrare research unit UMR-S951, Evry We recetly identified a signaling pathway that links the upregulation of miR-379 with a mitochondrial response in dystrophic muscle. In the present commentary, we explain the significance that this pathway may have in mitochondrial dysfunction in Duchenne muscular dystrophy (DMD). We identified the upregulation of miR-379 in the serum and muscles of DMD animal models and patients. We found that miR-379 is one of very few miRNAs whose expression was normalized in DMD patients treated with glucocorticoid. We identified EIF4G2 as an miR-379 target, which may promote mitochondrial oxidative phosphorylation (OxPhos) in the skeletal muscle. We found enriched EIF4G2 expression in oxidative fibers, and identified the mitochondrial ATP synthase subunit DAPIT as a translational target of EIF4G2. The identified signaling cascade, which comprises miR-379, EIF4G2 and DAPIT, may link the glucocorticoid treatment in DMD to a recovered mitochondrial ATP synthesis rate. We propose an updated model of mitochondrial dysfunction in DMD. https://pagepressjournals.org/index.php/bam/article/view/10012Duchenne Muscular DystrophyDLK1-DIO3miR-379EIF4G2DapitUSMG5 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Ai Vu Hong Mathilde Sanson Isabelle Richard David Israeli |
spellingShingle |
Ai Vu Hong Mathilde Sanson Isabelle Richard David Israeli A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy European Journal of Translational Myology Duchenne Muscular Dystrophy DLK1-DIO3 miR-379 EIF4G2 Dapit USMG5 |
author_facet |
Ai Vu Hong Mathilde Sanson Isabelle Richard David Israeli |
author_sort |
Ai Vu Hong |
title |
A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy |
title_short |
A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy |
title_full |
A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy |
title_fullStr |
A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy |
title_full_unstemmed |
A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy |
title_sort |
revised model for mitochondrial dysfunction in duchenne muscular dystrophy |
publisher |
PAGEPress Publications |
series |
European Journal of Translational Myology |
issn |
2037-7452 2037-7460 |
publishDate |
2021-09-01 |
description |
We recetly identified a signaling pathway that links the upregulation of miR-379 with a mitochondrial response in dystrophic muscle. In the present commentary, we explain the significance that this pathway may have in mitochondrial dysfunction in Duchenne muscular dystrophy (DMD). We identified the upregulation of miR-379 in the serum and muscles of DMD animal models and patients. We found that miR-379 is one of very few miRNAs whose expression was normalized in DMD patients treated with glucocorticoid. We identified EIF4G2 as an miR-379 target, which may promote mitochondrial oxidative phosphorylation (OxPhos) in the skeletal muscle. We found enriched EIF4G2 expression in oxidative fibers, and identified the mitochondrial ATP synthase subunit DAPIT as a translational target of EIF4G2. The identified signaling cascade, which comprises miR-379, EIF4G2 and DAPIT, may link the glucocorticoid treatment in DMD to a recovered mitochondrial ATP synthesis rate. We propose an updated model of mitochondrial dysfunction in DMD.
|
topic |
Duchenne Muscular Dystrophy DLK1-DIO3 miR-379 EIF4G2 Dapit USMG5 |
url |
https://pagepressjournals.org/index.php/bam/article/view/10012 |
work_keys_str_mv |
AT aivuhong arevisedmodelformitochondrialdysfunctioninduchennemusculardystrophy AT mathildesanson arevisedmodelformitochondrialdysfunctioninduchennemusculardystrophy AT isabellerichard arevisedmodelformitochondrialdysfunctioninduchennemusculardystrophy AT davidisraeli arevisedmodelformitochondrialdysfunctioninduchennemusculardystrophy AT aivuhong revisedmodelformitochondrialdysfunctioninduchennemusculardystrophy AT mathildesanson revisedmodelformitochondrialdysfunctioninduchennemusculardystrophy AT isabellerichard revisedmodelformitochondrialdysfunctioninduchennemusculardystrophy AT davidisraeli revisedmodelformitochondrialdysfunctioninduchennemusculardystrophy |
_version_ |
1717377039764291584 |