Studies using Drosophila models of C9ALS/FTD identified a vicious feedback cycle for DPR-mediated TDP-43 proteinopathy characterised by cytoplasmic TDP-43 and KPNA4 mislocalisation (Solomon et al., 2018), a phenotype that was also observed in post-mortem frontal cortex tissue of C9FTD and sporadic FTD-TDP cases (Solomon et al., 2018; Chou et al., 2018). The gene discussed is KPNA4; the disease is frontotemporal dementia.