Concerns have commonly been raised as to the relevance of DPRs in terms of mechanistic contribution to C9orf72‐ALS/FTD, due to the abundance and widespread DPR distribution throughout brain and spinal cord prior to neuronal cell death (Mackenzie et al, 2013, 2015; Gomez‐Deza et al, 2015). This evidence concerns the gene C9orf72 and amyotrophic lateral sclerosis.