PMID- 35081731
OWN - NLM
STAT- MEDLINE
DCOM- 20220804
LR  - 20220812
IS  - 1557-7716 (Electronic)
IS  - 1523-0864 (Linking)
VI  - 37
IP  - 4-6
DP  - 2022 Aug
TI  - Redox Signaling and Stress in Inherited Myopathies.
PG  - 301-323
LID - 10.1089/ars.2021.0266 [doi]
AB  - Significance: Reactive oxygen species (ROS) are highly reactive compounds that 
      behave like a double-edged sword; they damage cellular structures and act as 
      second messengers in signal transduction. Mitochondria and endoplasmic reticulum 
      (ER) are interconnected organelles with a central role in ROS production, 
      detoxification, and oxidative stress response. Skeletal muscle is the most 
      abundant tissue in mammals and one of the most metabolically active ones and thus 
      relies mainly on oxidative phosphorylation (OxPhos) to synthesize adenosine 
      triphosphate. The impairment of OxPhos leads to myopathy and increased ROS 
      production, thus affecting both redox poise and signaling. In addition, ROS enter 
      the ER and trigger ER stress and its maladaptive response, which also lead to a 
      myopathic phenotype with mitochondrial involvement. Here, we review the role of 
      ROS signaling in myopathies due to either mitochondrial or ER dysfunction. Recent 
      Advances: Relevant advances have been evolving over the last 10 years on the 
      intricate ROS-dependent pathways that act as modifiers of the disease course in 
      several myopathies. To this end, pathways related to mitochondrial biogenesis, 
      satellite cell differentiation, and ER stress have been studied extensively in 
      myopathies. Critical Issues: The analysis of the chemistry and the exact 
      quantitation, as well as the localization of ROS, are still challenging due to 
      the intrinsic labile nature of ROS and the technical limitations of their 
      sensors. Future Directions: The mechanistic studies of the pathogenesis of 
      mitochondrial and ER-related myopathies offer a unique possibility to discover 
      novel ROS-dependent pathways. Antioxid. Redox Signal. 37, 301-323.
FAU - Dogan, Sukru Anil
AU  - Dogan SA
AUID- ORCID: 0000-0002-1800-2923
AD  - Department of Molecular Biology and Genetics, Center for Life Sciences and 
      Technologies, Bogazici University, Istanbul, Turkey.
FAU - Giacchin, Giacomo
AU  - Giacchin G
AD  - Department of Biomedical Sciences, University of Padova, Padova, Italy.
FAU - Zito, Ester
AU  - Zito E
AD  - Department of Biomolecular Sciences, University of Urbino Carlo Bo, Urbino, 
      Italy.
AD  - Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Milan, Italy.
FAU - Viscomi, Carlo
AU  - Viscomi C
AUID- ORCID: 0000-0001-6050-0566
AD  - Department of Biomedical Sciences, University of Padova, Padova, Italy.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20220418
PL  - United States
TA  - Antioxid Redox Signal
JT  - Antioxidants & redox signaling
JID - 100888899
RN  - 0 (Reactive Oxygen Species)
SB  - IM
MH  - Animals
MH  - Endoplasmic Reticulum/metabolism
MH  - *Endoplasmic Reticulum Stress
MH  - Mammals/metabolism
MH  - *Muscular Diseases/genetics/metabolism
MH  - Oxidation-Reduction
MH  - Oxidative Stress
MH  - Reactive Oxygen Species/metabolism
MH  - Signal Transduction
OTO - NOTNLM
OT  - ER-related myopathies
OT  - mitochondrial myopathies
OT  - mitochondrial-ER contact sites
OT  - neuromuscular diseases
OT  - reactive oxygen species
OT  - satellite cells
OT  - unfolded protein response
EDAT- 2022/01/28 06:00
MHDA- 2022/08/05 06:00
CRDT- 2022/01/27 05:35
PHST- 2022/01/28 06:00 [pubmed]
PHST- 2022/08/05 06:00 [medline]
PHST- 2022/01/27 05:35 [entrez]
AID - 10.1089/ars.2021.0266 [doi]
PST - ppublish
SO  - Antioxid Redox Signal. 2022 Aug;37(4-6):301-323. doi: 10.1089/ars.2021.0266. Epub 
      2022 Apr 18.