The direct involvement of the hypothalamus has been hypothesized to be due to its role as the main regulator of energy homeostasis, feeding and satiety [52] and has been supported by evidence of reduced hypothalamic volume in ALS patients [19, 20] and the detection of ALS-related TDP-43 pathology in the hypothalamus of a subset of ALS patients [53, 54]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.