Skeletal Muscle in ALS: An Unappreciated Therapeutic Opportunity?

Silvia Scaricamazza, Illari Salvatori, Alberto Ferri, Cristiana Valle

Research output: Contribution to journalReview articlepeer-review

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the selective degeneration of upper and lower motor neurons and by the progressive weakness and paralysis of voluntary muscles. Despite intense research efforts and numerous clinical trials, it is still an incurable disease. ALS had long been considered a pure motor neuron disease; however, recent studies have shown that motor neuron protection is not sufficient to prevent the course of the disease since the dismantlement of neuromuscular junctions occurs before motor neuron degeneration. Skeletal muscle alterations have been described in the early stages of the disease, and they seem to be mainly involved in the "dying back" phenomenon of motor neurons and metabolic dysfunctions. In recent years, skeletal muscles have been considered crucial not only for the etiology of ALS but also for its treatment. Here, we review clinical and preclinical studies that targeted skeletal muscles and discuss the different approaches, including pharmacological interventions, supplements or diets, genetic modifications, and training programs.

Original languageEnglish
JournalCells
Volume10
Issue number3
DOIs
Publication statusPublished - Mar 2 2021

Keywords

  • amyotrophic lateral sclerosis
  • genetic intervention
  • pharmacological approaches
  • physical activity
  • skeletal muscle

ASJC Scopus subject areas

  • Medicine(all)

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