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Deficient Sarcolemma Repair in ALS: A Novel Mechanism with Therapeutic Potential

Li, Ang
; 
Yi, Jianxun
; 
Li, Xuejun
; 
Dong, Li
; ; 
Ma, Jianjie
; 
Zhou, Jingsong
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Genre
Journal article
Date
2022-10-17
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Department
Neurology
Subject
Sarcolemma permeability
 MG53
 Membrane repair
 ROS
 ALS
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http://hdl.handle.net/20.500.12613/9967
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OstrowEtAl-JournalArticle-2022-10.pdf
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http://dx.doi.org/10.3390/cells11203263
Abstract
The plasma membrane (sarcolemma) of skeletal muscle myofibers is susceptible to injury caused by physical and chemical stresses during normal daily movement and/or under disease conditions. These acute plasma membrane disruptions are normally compensated by an intrinsic membrane resealing process involving interactions of multiple intracellular proteins including dysferlin, annexin, caveolin, and Mitsugumin 53 (MG53)/TRIM72. There is new evidence for compromised muscle sarcolemma repair mechanisms in Amyotrophic Lateral Sclerosis (ALS). Mitochondrial dysfunction in proximity to neuromuscular junctions (NMJs) increases oxidative stress, triggering MG53 aggregation and loss of its function. Compromised membrane repair further worsens sarcolemma fragility and amplifies oxidative stress in a vicious cycle. This article is to review existing literature supporting the concept that ALS is a disease of oxidative-stress induced disruption of muscle membrane repair that compromise the integrity of the NMJs and hence augmenting muscle membrane repair mechanisms could represent a viable therapeutic strategy for ALS.
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Citation
Li, A.; Yi, J.; Li, X.; Dong, L.; Ostrow, L.W.; Ma, J.; Zhou, J. Deficient Sarcolemma Repair in ALS: A Novel Mechanism with Therapeutic Potential. Cells 2022, 11, 3263. https://doi.org/10.3390/cells11203263
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MDPI
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Cells, Vol. 11, Iss. 20
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