Senataxin is mutated in juvenile forms of Ataxia and amyotrophic lateral sclerosis (ALS) (Chen et al., 2004; Moreira et al., 2004), while RNaseH2 in the neuroinflammatory Aicardi–Goutières disorder (Crow et al., 2006). The gene discussed is SETX; the disease is amyotrophic lateral sclerosis.