The dysfunction of another protein in this family, CHCHD10 (Figure 1B), emerged recently as a relevant factor in neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS) and ALS-frontotemporal dementia (ALS-FTLD) [112,113,114], among other neurodegenerative diseases. Here, CHCHD10 is linked to frontotemporal dementia.