,16 These studies provide experimental evidence that accumulating cytosolic TDP-43 causes cytoplasmic mislocalization of KPNAs in Drosophila models of C9ALS/FTD, a phenotype that is also observed in post-mortem frontal cortex tissue of C9FTD patients and sporadic cases of FTD-TDP.15 This evidence concerns the gene TARDBP and frontotemporal dementia.