In this section, we provide a literature review of the last five years on the connection between mitochondrial impairment and the most common proteins involved in ALS onset—namely, SOD1, TDP-43, C9ORF72, FUS, and CHCHD10, together with a grouped paragraph for the other genes involved (Table 1). Here, CHCHD10 is linked to amyotrophic lateral sclerosis.