Autophagy and mitophagy in ALS

Amyotrophic lateral sclerosis (ALS) is a debilitating and incurable disease involving the loss of motor neurons and subsequent muscle atrophy. Genetic studies have implicated deficits in autophagy and/or mitophagy in the onset of the disease. Here we review recent progress in our understanding of th...

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Bibliographic Details
Published in:Neurobiology of disease 2019-02, Vol.122, p.35-40
Main Authors: Evans, Chantell S., Holzbaur, Erika L.F.
Format: Article
Language:English
Subjects:
ALS
Amyotrophic Lateral Sclerosis - physiopathology
Animals
Autophagy
Autophagy - physiology
Humans
Mitophagy
Mitophagy - physiology
Motor neuron disease
Neurons - physiology
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Summary:Amyotrophic lateral sclerosis (ALS) is a debilitating and incurable disease involving the loss of motor neurons and subsequent muscle atrophy. Genetic studies have implicated deficits in autophagy and/or mitophagy in the onset of the disease. Here we review recent progress in our understanding of the pathways for autophagy and mitophagy in neurons, and how these pathways may be affected by mutations in genes including DCTN1, OPTN, TBK1, VCP, and C9ORF72. We also discuss the implications of modulating autophagy in ALS, highlighting both the potential of the approach and the concerns raised by targeting this pathway as a therapeutic strategy in neurodegenerative disease.
ISSN:0969-9961
1095-953X
DOI:10.1016/j.nbd.2018.07.005