Despite similar expression levels of DHX30 in mitochondria of WT and P525L FUS, cells expressing FUS mutants showed reduced immunoreactivity against the anti-DHX30 antibody in IF analysis, which was the same as in spinal motor neurons of ALS-FUS by the immunoelectron microscope (Fig. 2c,e). Here, FUS is linked to amyotrophic lateral sclerosis.