Epilepsy-associated E344Q mutation in importin alpha-7 reduces its binding to NLSs, limiting importin alpha-7 binding to both hnRNP R and hnRNP U in iPSCs-derived neurons. Mutations in the hnRNP R bipartite NLS are associated with brain and cerebellar abnormalities. Here, KPNA6 is linked to epilepsy.