Studies in experimental models have found increased hnRNPA1 transcript and cytoplasmic hnRNPA1 protein levels in TDP-43 depleted cells.28 Consistent with this, and previous findings of cytoplasmic hnRNPA1 in post-mortem ALS cases,29,30 we demonstrate significant nuclear depletion accompanied by cytoplasmic immunoreactivity of hnRNPA1 in the frontal cortex, which is the predilection site of TDP-43 dysfunction in FTLD and FTLD-ALS cases. Here, TARDBP is linked to amyotrophic lateral sclerosis.