Distinct roles for motor neuron autophagy early and late in the SOD1 G93A mouse model of ALS

Abstract excerpt

Significance Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that leads to death of spinal motor neurons and paralysis. Genetic studies of ALS patients have identified mutations in autophagy pathway genes including p62 / SQSTM1 , OPTN , TBK1 , VCP , and C9ORF72 . However, the mechanisms by which these mutations cause ALS are not understood. Here we investigated the role of autophagy in a mouse...

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