For this purpose, we utilized wild type (WT) and CHCHD10 transgenic mouse variants, in which N-terminally Flag-tagged wild type (CHCHD10WT) or FTD/ALS-linked mutants (CHCHD10R15L or CHCHD10S59L) are expressed in the central nervous system (CNS) under control of the neuron-specific mouse PrP promoter [26]. This evidence concerns the gene CHCHD10 and amyotrophic lateral sclerosis.