These include mis-accumulated phosphorylated TDP-43, SOD1 aggregates and dipeptide repeats (DPRs) found in the most common inherited form of ALS, due to a repeat expansion mutation in C9ORF72. TDP-43 proteinopathy is known to be the most prevalent and has been implicated in many pathways, including mitochondrial dysfunction, autophagy dysregulation and impaired endocytosis [153]. Here, TARDBP is linked to amyotrophic lateral sclerosis.