Whilst ALS, FTD and IBM pathology are distinct in each tissue and can exist independently, they share common pathological hallmarks which are indicative of protein dyshomeostasis, with the presence of ubiquitin-positive inclusion bodies, p62 aggregation and mislocalisation of the RNA-binding protein, TDP-43, observed in all three disorders [9–11] . This evidence concerns the gene TARDBP and frontotemporal dementia.