Given the modifying effect on C9orf72-associated disease pathogenesis, and our discovery that C9orf72 patients display reduced levels of RuvBL1/2 expression (Fig 2), we propose that modulating RuvBL1/2 levels could be beneficial in alleviating DPR-associated disease mechanisms in C9orf72-ALS/FTD. This evidence concerns the gene C9orf72 and frontotemporal dementia.