Mitochondrial Dysfunction as an Underlying Cause of Skeletal Muscle Disorders

Chen, Tsung‐Hsien; Koh, Kok-Yean; Lin, Keh Ming; Chou, Chia-Hung

doi:10.3390/ijms232112926

Cited by 27 publications

(14 citation statements)

References 173 publications

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“…Muscle humanin expression is upregulated in humans, in response to exercise stress, mtDNA mutation-associated diseases, and healthy aging, suggesting a tissue-specific response aimed at restoring mitochondrial homeostasis [ 102 ] in response to stress from, for example, acute exercise [ 102 ]. Several skeletal muscle diseases are manifested by either an excess of leading to atrophy, or a reduced autophagy mechanism leading to muscle and mitochondrial degeneration [ 103 ].…”

Section: Resultsmentioning

confidence: 99%

Humanin and Its Pathophysiological Roles in Aging: A Systematic Review

Coradduzza

Congiargiu

Chen

et al. 2023

Biology

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Background: Senescence is a cellular aging process in all multicellular organisms. It is characterized by a decline in cellular functions and proliferation, resulting in increased cellular damage and death. These conditions play an essential role in aging and significantly contribute to the development of age-related complications. Humanin is a mitochondrial-derived peptide (MDP), encoded by mitochondrial DNA, playing a cytoprotective role to preserve mitochondrial function and cell viability under stressful and senescence conditions. For these reasons, humanin can be exploited in strategies aiming to counteract several processes involved in aging, including cardiovascular disease, neurodegeneration, and cancer. Relevance of these conditions to aging and disease: Senescence appears to be involved in the decay in organ and tissue function, it has also been related to the development of age-related diseases, such as cardiovascular conditions, cancer, and diabetes. In particular, senescent cells produce inflammatory cytokines and other pro-inflammatory molecules that can participate to the development of such diseases. Humanin, on the other hand, seems to contrast the development of such conditions, and it is also known to play a role in these diseases by promoting the death of damaged or malfunctioning cells and contributing to the inflammation often associated with them. Both senescence and humanin-related mechanisms are complex processes that have not been fully clarified yet. Further research is needed to thoroughly understand the role of such processes in aging and disease and identify potential interventions to target them in order to prevent or treat age-related conditions. Objectives: This systematic review aims to assess the potential mechanisms underlying the link connecting senescence, humanin, aging, and disease.

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Section: Resultsmentioning

confidence: 99%

Humanin and Its Pathophysiological Roles in Aging: A Systematic Review

Coradduzza

Congiargiu

Chen

et al. 2023

Biology

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“…Mitochondrial biology in CARM1 mKO mice. Following this, we explored the expression of several molecules that are important in mitochondrial health (34,35). Exercise increased (P < 0.05) protein content of PGC-1α and OPA1 only in F animals, whereas levels of BNIP3, FIS1, sum of representative subunits of complex 1 (CI)-CV of mitochondrial OXPHOS, and CS were similar between SED and VWR mice (Fig.…”

Section: Resultsmentioning

confidence: 99%

Sex-Specific Effect of CARM1 in Skeletal Muscle Adaptations to Exercise

VANLIESHOUT,

STOUTH,

RAZIEE

et al. 2023

Medicine &Amp; Science in Sports &Amp; Exercise

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Purpose The purpose of this study was to determine how the intersection of coactivator-associated arginine methyltransferase 1 (CARM1) and biological sex impacts skeletal muscle adaptations to chronic physical activity. Methods 12-week-old female (F) and male (M) wild-type (WT) and CARM1 skeletal muscle-specific knockout mice (mKO) were randomly assigned to sedentary (SED) or voluntary wheel running (VWR) experimental groups. For 8 weeks, the animals in the VWR cohort had volitional access to running wheels. Subsequently, we performed whole-body functional tests, and 48 hours later muscles were harvested for molecular analysis. Western blotting, enzyme activity assays, as well as confocal and transmission electron microscopy (TEM) were used to examine skeletal muscle biology. Results Our data reveal a sex-dependent reduction in VWR volume caused by muscle-specific ablation of CARM1, as F CARM1 mKO mice performed less chronic, volitional exercise than their WT counterparts. Regardless of VWR output, exercise-induced adaptations in physiological function were similar between experimental groups. A broad panel of protein arginine methyltransferase (PRMT) biology measurements, including markers of arginine methyltransferase expression and activity, were unaffected by VWR, except for CARM1 and PRMT7 protein levels, which decreased and increased with VWR, respectively. Changes in myofiber morphology and mitochondrial protein content showed similar trends among animals. However, a closer examination of TEM images revealed contrasting responses to VWR in CARM1 mKO mice compared to WT littermates, particularly in mitochondrial size and fractional area. Conclusions The present findings demonstrate that CARM1 mKO reduces daily running volume in F mice, as well as exercise-evoked skeletal muscle mitochondrial plasticity, which indicates that this enzyme plays an essential role in sex-dependent differences in exercise performance and mitochondrial health.

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“…Further, microtubule disorganization has been noted in dystrophin-deficient mouse muscle which was associated with impaired ADP control of mitochondrial bioenergetics ( Ramos et al, 2020 ). While a low-level production of reactive oxygen species (ROS) is required for cell-cell signaling in normal cells, dysfunctional mitochondria in dystrophic muscle release abnormal levels of ROS, which can then activate pro-inflammatory pathways which further exacerbate disease pathology ( Chen et al, 2022b ). Further, it has been shown using nuclear magnetic resonance spectroscopy, that such elevated ROS production and damaged mitochondrial membrane in human and mouse dystrophic mitochondria are associated with reduced ATP generation and oxidative capacity ( Kuznetsov et al, 1998 ; Griffin et al, 2001 ; Sharma et al, 2003 ).…”

Section: Mitochondrial Dysfunctionmentioning

confidence: 99%

Satellite cell contribution to disease pathology in Duchenne muscular dystrophy

Kodippili

Rudnicki

2023

Front. Physiol.

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Progressive muscle weakness and degeneration characterize Duchenne muscular dystrophy (DMD), a lethal, x-linked neuromuscular disorder that affects 1 in 5,000 boys. Loss of dystrophin protein leads to recurrent muscle degeneration, progressive fibrosis, chronic inflammation, and dysfunction of skeletal muscle resident stem cells, called satellite cells. Unfortunately, there is currently no cure for DMD. In this mini review, we discuss how satellite cells in dystrophic muscle are functionally impaired, and how this contributes to the DMD pathology, and the tremendous potential of restoring endogenous satellite cell function as a viable treatment strategy to treat this debilitating and fatal disease.

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Mitochondrial Dysfunction as an Underlying Cause of Skeletal Muscle Disorders

Cited by 27 publications

References 173 publications

Humanin and Its Pathophysiological Roles in Aging: A Systematic Review

Humanin and Its Pathophysiological Roles in Aging: A Systematic Review

Sex-Specific Effect of CARM1 in Skeletal Muscle Adaptations to Exercise

Satellite cell contribution to disease pathology in Duchenne muscular dystrophy

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