Other more-severe CHCHD10 variants are a rare cause of dominant amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (Bannwarth et al., 2014; Müller et al., 2014), and of mitochondrial myopathy (Ajroud-Driss et al., 2015), which has led to intense investigations of the underlying disease mechanisms. This evidence concerns the gene CHCHD10 and amyotrophic lateral sclerosis.