TDP-43 has recently drawn considerable attention because it has been found that hyperphosphorylation-associated aggregation of TDP-43 is a signature of two neurodegenerative diseases: amyotrophic sclerosis (ALS) and frontotemporal lobar degeneration (FTLD-TDP) [89, 90]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.