Both OPTN and TBK1 mutants have TDP-43 proteinopathy, and the OPTN protein is incorporated into pathological aggregates present in several neurodegenerative diseases, including the DPR inclusions specific to C9ORF72-ALS/FTD (Bury et al., 2016). The gene discussed is C9orf72; the disease is frontotemporal dementia.