In 2006, the transactive response (TAR) DNA-binding protein (TDP-43) was identified as the main component of intraneuronal cytosolic ubiquitinated protein aggregates found in ALS and tau-negative, ubiquitin-positive frontotemporal lobar degeneration (FTLD) patients [1,2]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.