In sporadic ALS and some familial ALS, including C9ORF72- and TARDBP-associated ALS, loss of transactive response element DNA/RNA binding protein 43 (TDP-43) in the nucleus and abnormal TDP-43 positive cytoplasmic inclusions (TDP-43 pathology) in motor neurons are observed [3,4,5,6]. Here, TARDBP is linked to amyotrophic lateral sclerosis.