Conversely, whereas WT CHCHD10 fully retained TDP-43 localization to the nucleus, FTD/ALS-associated CHCHD10 mutations (R15L and S59L) induced cytoplasmic mislocalization of TDP-43 in irregularly shaped inclusions often co-localized with mitochondria. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.