More than 50 disease-associated mutations of TDP-43 gene have been identified in amyotrophic lateral sclerosis (ALS) or frontotemporal lobar degeneration (FTLD) patients, which are mainly concentrated in the glycine-rich C-terminal region, indicating that the ability of TDP-43 for cooperative assembly on RNA binding sites plays a role in disease mechanisms. Here, TARDBP is linked to amyotrophic lateral sclerosis.