Journal
CELLS
Volume 10, Issue 3, Pages -Publisher
MDPI
DOI: 10.3390/cells10030525
Keywords
amyotrophic lateral sclerosis; skeletal muscle; pharmacological approaches; physical activity; genetic intervention
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Funding
- AriSLA through the HyperALS project
- AFM-Telethon project [2018, 21021]
- Ministry of Health, Italy-United States of America. 2020-Whole transcriptome analysis in models of extended healthy lifespan after spermidine treatment [PGR01040]
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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by motor neuron degeneration and muscle weakness. Despite efforts to target motor neurons, skeletal muscle alterations have been found to play a crucial role in the progression of the disease.
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.
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