TDP-43 is a major protein component in ubiquitin-positive, tau- and α-synuclein-negative inclusions of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) [1], [2], which was initially identified due to its specific enrichment in the detergent-insoluble biochemical fraction of FTLD frontal cortex [1]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.