Despite multiple functional overlaps and 54% protein sequence identity between CHCHD2 and CHCHD10, rare mutations in CHCHD2 are associated with PD and other LBDs (Funayama et al., 2015; Ogaki et al., 2015), whereas mutations in CHCHD10 are associated with the spectrum of amyotrophic lateral sclerosis (ALS)-frontotemporal dementia (FTD) (Bannwarth et al., 2014; Johnson et al., 2014; Müller et al., 2014; Zhang et al., 2015). This evidence concerns the gene CHCHD2 and frontotemporal dementia.