C9orf72 and amyotrophic lateral sclerosis: Here, we demonstrate that overexpression of RuvBL1, but predominantly RuvBL2, are able to reduce C9orf72-associated DPR levels in a range of in vitro models including cell lines, primary neurons, and patient-derived iPSC neuron cells, as well as an in vivo Drosophila model of C9orf72-ALS/FTD (Figs 1, 3–5).