Specifically, FTD/ALS-linked CHCHD10R15L and CHCHD10S59L promote cytoplasmic and mitochondrial accumulation of misfolded TDP-43 [22,23], whereas wild-type CHCHD10 (CHCHD10WT) mitigates TDP-43 aggregation and TDP-43-induced mitochondrial dysfunction [20,22,23,24]. Here, TARDBP is linked to frontotemporal dementia.