Accumulation of TDP-43 aggregates in the central nervous system is a common feature of many neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS), FTD, Alzheimer’s disease, and limbic-predominant age-related TDP-43 encephalopathy with dementia [52, 53]. The gene discussed is TARDBP; the disease is early-onset autosomal dominant Alzheimer disease.