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

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

Research output: Contribution to journalReview articlepeer-review


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.

Original languageEnglish (US)
Article number3263
Issue number20
StatePublished - Oct 2022


  • ALS
  • MG53
  • ROS
  • membrane repair
  • sarcolemma permeability

ASJC Scopus subject areas

  • General Biochemistry, Genetics and Molecular Biology


Dive into the research topics of 'Deficient Sarcolemma Repair in ALS: A Novel Mechanism with Therapeutic Potential'. Together they form a unique fingerprint.

Cite this