Hence it is possible that stress granule assembly promotes an environment for poly-GR to aggregate, undergo aberrant interactions (such as with TDP-43) and enhance toxicity, therefore modulating this process via reducing ataxin-2 levels may provide a novel therapeutic target in C9orf72-ALS/FTD (Figure 3, panel 6). This evidence concerns the gene ATXN2 and frontotemporal dementia.