Indeed, cellular co-localization of the arginine-containing DPR proteins with nucleoli20,22,39 and mitochondrial components24,39,40, and the extracellular localization of these DPR proteins22 demonstrate that C9ORF72-ALS/FTD-associated poly-PR and poly-GR can be liberated from ribosomes into the cytoplasm and organelles, and can impair g'lobal translation in trans. This evidence concerns the gene C9orf72 and amyotrophic lateral sclerosis.