Mitoautophagy (a self-destructive path involving mitochondria’s elongation and the formation of a ring-like structure before its disintegration) was found in upper MNs of ALS mice, particularly those with TARDBP or PFN1 mutations, supporting an association between ALS with TDP-43 toxic aggregates and mitochondrial dysfunction [85]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.