Given that TDP-43 influences, directly and indirectly, fundamental cellular processes, it is not surprising that its dysfunction contributes to neurodegenerative diseases, including ALS, FTD and ALS-FTD, but also Alzheimer’s disease, Parkinson’s disease (PD), dementia with Lewy bodies (DLB), and limbic predominant age-related TDP-43 encephalopathy (LATE) [161,162,163,164]. The gene discussed is TARDBP; the disease is Lewy body dementia.