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

Li, Ang; Yi, Jianxun; Li, Xuejun; Dong, Li; Ostrow, Lyle W.; Ma, Jianjie; Zhou, Jingsong

doi:10.3390/cells11203263

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

by

Ang Li

¹

,

Jianxun Yi

¹,

Xuejun Li

¹,

Li Dong

¹,

Lyle W. Ostrow

^2,*,

Jianjie Ma

^3,* and

Jingsong Zhou

^1,*

¹

Department of Kinesiology, College of Nursing and Health Innovation, University of Texas at Arlington, Arlington, TX 76019, USA

²

Department of Neurology, Lewis Katz School of Medicine at Temple University, Philadelphia, PA 19122, USA

³

Department of Surgery, University of Virginia, Charlottesville, VA 22903, USA

^*

Authors to whom correspondence should be addressed.

Cells 2022, 11(20), 3263; https://doi.org/10.3390/cells11203263

Submission received: 6 September 2022 / Revised: 7 October 2022 / Accepted: 14 October 2022 / Published: 17 October 2022

(This article belongs to the Special Issue Redox Control of Cell Signaling in Cardiac and Skeletal Muscle)

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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.

Keywords: sarcolemma permeability; MG53; membrane repair; ROS; ALS

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MDPI and ACS Style

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

AMA Style

Li A, Yi J, Li X, Dong L, Ostrow LW, Ma J, Zhou J. Deficient Sarcolemma Repair in ALS: A Novel Mechanism with Therapeutic Potential. Cells. 2022; 11(20):3263. https://doi.org/10.3390/cells11203263

Chicago/Turabian Style

Li, Ang, Jianxun Yi, Xuejun Li, Li Dong, Lyle W. Ostrow, Jianjie Ma, and Jingsong Zhou. 2022. "Deficient Sarcolemma Repair in ALS: A Novel Mechanism with Therapeutic Potential" Cells 11, no. 20: 3263. https://doi.org/10.3390/cells11203263

APA Style

Li, A., Yi, J., Li, X., Dong, L., Ostrow, L. W., Ma, J., & Zhou, J. (2022). Deficient Sarcolemma Repair in ALS: A Novel Mechanism with Therapeutic Potential. Cells, 11(20), 3263. https://doi.org/10.3390/cells11203263

Note that from the first issue of 2016, this journal uses article numbers instead of page numbers. See further details here.

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

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