While CHCHD10R15L and CHCHD10S59L are rare mutations found in familial ALS–FTD patients, endogenous CHCHD10 is significantly downregulated in brains of TDP-43 transgenic mice and sporadic ALS and FTD patients who do not carry CHCHD10 mutations [20,24,48]. Here, CHCHD10 is linked to frontotemporal dementia.