Impaired Mitophagy Plays a Role in Denervation of Neuromuscular Junctions in ALS Mice.

ABSTRACT: Motor neurons in amyotrophic lateral sclerosis (ALS) patients and animal models show degeneration from the nerve terminal, known as dying-back neuropathy. To investigate the mechanism underlying this neuropathy, we analyzed the neuromuscular junctions (NMJs) and motor neuron cell bodies in SOD1^G93A mice using electron microscopy. NMJs of SOD1^G93A mice exhibited significantly higher numbers of autophagosomes and degenerated mitochondria compared to wild-type controls. Mitophagosomes were identified in the NMJ presynaptic terminals of wild-type mice and SOD1^G93A mice. However, the number of mitophagosomes did not increase significantly in SOD1^G93A NMJs indicating a defect in mitophagy, the autophagic process to degrade mitochondria. Consistent with

SUBMITTER: Rogers RS 

PROVIDER: S-EPMC5575151 | biostudies-literature | 2017

REPOSITORIES: biostudies-literature