The neuropathological process underlying amyotrophic lateral sclerosis (ALS) entails abnormal changes of the endogenous and predominantly intranuclear protein TDP-43 (transactive response DNA-binding protein 43) which progress at different rates, but in a similar sequence of four stages, in ALS patients’ brains [1, 2]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.