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

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 mice using electron microscopy. NMJs of SOD1 mice exhibited significantly higher numbers of autophagosomes and degenerated mitochondria compared to wild-type controls.