The presence of abnormal forms of TDP-43, such as TDP-43 mutations and aggregates in the central nervous system, is a common feature of several neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), Alzheimer’s disease (AD), and limbic predominant age-related TDP-43 encephalopathy (LATE). The gene discussed is TARDBP; the disease is frontotemporal dementia.