Inversely, RNA-seq data analysis of sporadic ALS spinal cords revealed a significant downregulation of KPNB1 [84], suggesting that cytoplasmic mislocalization or reduced levels of NIRs in ALS/FTD can impede TDP-43 nuclear import and thus facilitate its pathological mislocalization. This evidence concerns the gene KPNB1 and amyotrophic lateral sclerosis.