Several studies have aimed to identify the RBPs that are sequestered within the C9orf72 RNA foci and have found hnRNPA1/3, PUR-a, ADARB2, Nucleolin, SRSF2, and ALYREF in post mortem CNS tissue, iPSCs-derived cortical neurons reprogrammed from C9orf72-related ALS-FTD patient fibroblasts, neuronal cell lines or a Drosophila model of C9orf72 ALS (Donnelly et al., 2013; Sareen et al., 2013; Cooper-Knock et al., 2014; Hautbergue et al., 2017). This evidence concerns the gene C9orf72 and frontotemporal dementia.