Dysfunction of TDP-43 is frequently observed in human neurodegenerative disease, particularly in brain tissues from patients with amyotrophic lateral sclerosis and frontotemporal dementia, Parkinson’s disease, and limbic-predominant age-related TDP-43 encephalopathy (1, 2, 3, 4, 5). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.