However, although overexpressed FUS has been shown to localize in mitochondria and abnormalities/damages to mitochondria are observed in FUS-ALS and FUS-FTD29–31, its specific functions in mitochondria, particularly with respect to mitochondrial genome maintenance, and its overall native physiological role in mitochondria remain largely uncharacterized. Here, FUS is linked to amyotrophic lateral sclerosis.