PMID- 34533019
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20211026
IS  - 2037-7452 (Print)
IS  - 2037-7460 (Electronic)
IS  - 2037-7452 (Linking)
VI  - 31
IP  - 3
DP  - 2021 Sep 17
TI  - A revised model for mitochondrial dysfunction in Duchenne muscular dystrophy.
LID - 10.4081/ejtm.2021.10012 [doi]
LID - 10012
AB  - We recently 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 a 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.
FAU - Vu Hong, Ai
AU  - Vu Hong A
AD  - Genethon, Evry, France; Universite Paris-Saclay, Univ Evry, Inserm, Genethon, 
      Integrare research unit UMR-S951, Evry. avuhong@genethon.fr.
FAU - Sanson, Mathilde
AU  - Sanson M
AD  - Genethon, Evry, France; Universite Paris-Saclay, Univ Evry, Inserm, Genethon, 
      Integrare research unit UMR-S951, Evry. mathildesanson199@gmail.com.
FAU - Richard, Isabelle
AU  - Richard I
AD  - Genethon, Evry, France; Universite Paris-Saclay, Univ Evry, Inserm, Genethon, 
      Integrare research unit UMR-S951, Evry. richard@genethon.fr.
FAU - Israeli, David
AU  - Israeli D
AD  - Genethon, Evry, France; Universite Paris-Saclay, Univ Evry, Inserm, Genethon, 
      Integrare research unit UMR-S951, Evry. israeli@genethon.fr.
LA  - eng
PT  - Journal Article
DEP - 20210917
PL  - Italy
TA  - Eur J Transl Myol
JT  - European journal of translational myology
JID - 101576208
PMC - PMC8495359
COIS- We confirm that we have read the Journal's position on issues involved in ethical 
      publication and affirm that this report is consistent with those guidelines.
EDAT- 2021/09/18 06:00
MHDA- 2021/09/18 06:01
PMCR- 2021/09/27
CRDT- 2021/09/17 07:53
PHST- 2021/08/03 00:00 [received]
PHST- 2021/09/12 00:00 [accepted]
PHST- 2021/09/18 06:00 [pubmed]
PHST- 2021/09/18 06:01 [medline]
PHST- 2021/09/17 07:53 [entrez]
PHST- 2021/09/27 00:00 [pmc-release]
AID - 10.4081/ejtm.2021.10012 [doi]
PST - epublish
SO  - Eur J Transl Myol. 2021 Sep 17;31(3):10012. doi: 10.4081/ejtm.2021.10012.