In ALS specifically, there is accumulating evidence in both mouse and Drosophila models that degeneration of the NMJ, accompanied by mitochondrial abnormalities, is an early pre-symptomatic disease event21–25 FUS has been shown to interact with mitochondrial proteins (HSP60; ATP synthase β-subunit) and mutations in FUS which increase cytoplasmic FUS have been associated with mitochondrial fragmentation within neurons26,27. This evidence concerns the gene FUS and amyotrophic lateral sclerosis.