In the latter cases, it has been reported that glycine/arginine-rich (poly(GR)) proteins, one of the DPR species resulting from the RAN translation of the transcripts from C9orf72 G4C2 repeat expansion, can sequester cytoplasmic TDP-43 and promote its aggregation; this finding was further supported by the observation that neurons of C9orf72 ALS-FTD patients present pathological inclusions containing both poly(GR) and TDP-43 [57,58]. Here, TARDBP is linked to frontotemporal dementia.