Importantly, in contrast to hnRNPD and C, these three hnRNPs have been previously studied in tissue from patients with ALS and FTD and consistent with our current findings in the motor cortex, normal nuclear localisation of hnRNPA2B1 and H2 but not A1 were also identified in the lower motor neurons of ALS.29-31,38 Future studies in experimental models will be needed to determine the mechanistic involvement and interaction of these nuclear hnRNPs in ALS and FTLD. Here, HNRNPA2B1 is linked to frontotemporal dementia.