Year: 2022
Type: review
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.
Source: Cells
Authors: Ang Li, Jianxun Yi, Xuejun Li, Li Dong, Lyle W. Ostrow, Jianjie Ma, Jingsong Zhou
Language: English
Subfield: Clinical Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,303
Funders: U.S. Department of Defense, ALS Association, Target ALS, National Institutes of Health, Centers for Disease Control and Prevention, National Institute on Aging, National Heart, Lung, and Blood Institute, National Institute of Neurological Disorders and Stroke, National Institute of Arthritis and Musculoskeletal and Skin Diseases
Awards: Target microbiota to alleviate ALS progression, RESTORING NEUROMUSCULAR JUNCTION INTEGRITY TO ALLEVIATE ALS PROGRESSION, G2985789923, G3208791448, Ca signaling cross-talk from SR to mitochondria in heart muscle, G3715836397, G3897908939, G4000312203, G4741119045, G4745198457, G5115817091, Preserving Mitochondrial Function for Alleviating ALS Progression, G5281337781, G5483127057, G5506105317, Ca Signaling in Progression of Amyotrophic Lateral Sclerosis in Skeletal Muscle, G7195988390, G7406827670, G7953195446, G8653999069, G8852305619, Mitigating neuroinflammation and enhancing neuronal integrity in Alzheimer's disease, MG53 function in muscle aging, Multifaceted role of MG53 in alleviating neuromuscular function decline in ALS
OpenAlex ID: W4306651306