SETX and frontotemporal dementia: Subsequent investigations revealed that several ALS/FTD-linked genes including FUS, TDP-43, SOD1, C9orf72, Never-in-mitosis A (NIMA) related kinase-1 (NEK1), C21orf2, Senetaxin (SETX), and Valosin containing protein 1 (VCP) are involved in the DNA damage response (DDR; Acs et al., 2011; Fang et al., 2015; Guerrero et al., 2016; van Rheenen et al., 2016; Higelin et al., 2018; Konopka and Atkin, 2018; Wang H. et al., 2018; Bordoni et al., 2019; Mitra et al., 2019).