In addition to being the hallmark lesions in ALS and FTLD-TDP, inclusions containing TDP-43 are varyingly present in some other neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease, dementia with Lewy bodies, Huntington's disease, and chronic traumatic encephalopathy (CTE) [8]–[12], where the severity of TDP-43 pathologic change is associated with the rate of cognitive decline in affected patients [13]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.