According to the recent reports, apoptotic cell death of motor neurons (including SOD1, TDP-43, and FUS) [2–9, 54] and abnormal RNA metabolism (mRNA transcription and miRNAs) [9, 14, 55] in ALS are so controversial issues because non-apoptotic features have been found in ALS patients [54]. Here, TARDBP is linked to amyotrophic lateral sclerosis.