期刊
CELLS
卷 11, 期 3, 页码 -出版社
MDPI
DOI: 10.3390/cells11030415
关键词
amyotrophic lateral sclerosis; skeletal muscle; ion channels; animal models; clinical trials; therapy
类别
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease characterized by progressive loss of motor neurons and muscle function. The disease involves a complex cascade of events among motor neurons, glia, and muscles, and is associated with mitochondrial dysfunction and other mechanisms. Currently, there are limited treatment options, and there is a need to find effective treatments.
Amyotrophic Lateral Sclerosis is a neurodegenerative disease caused by progressive loss of motor neurons, which severely compromises skeletal muscle function. Evidence shows that muscle may act as a molecular powerhouse, whose final signals generate in patients a progressive loss of voluntary muscle function and weakness leading to paralysis. This pathology is the result of a complex cascade of events that involves a crosstalk among motor neurons, glia, and muscles, and evolves through the action of converging toxic mechanisms. In fact, mitochondrial dysfunction, which leads to oxidative stress, is one of the mechanisms causing cell death. It is a common denominator for the two existing forms of the disease: sporadic and familial. Other factors include excitotoxicity, inflammation, and protein aggregation. Currently, there are limited cures. The only approved drug for therapy is riluzole, that modestly prolongs survival, with edaravone now waiting for new clinical trial aimed to clarify its efficacy. Thus, there is a need of effective treatments to reverse the damage in this devastating pathology. Many drugs have been already tested in clinical trials and are currently under investigation. This review summarizes the already tested drugs aimed at restoring muscle-nerve cross-talk and on new treatment options targeting this tissue.
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