Genetic mutations linked with TDP-43 proteinopathies and ALS/FTD, including mutations to TARDBP, C9orf72, ataxin 2 (ATXN2) and superoxide dismutase 1 (SOD1), may confer vulnerability to caspase-cleavage of TDP-43. The gene discussed is ATXN2; the disease is frontotemporal dementia.