NR0B1 and alternating hemiplegia of childhood: On the other hand, it has been shown that DAX-1 C-terminal domain contains a potent transcriptional repressor domain that, when altered by mutations in AHC patients, impairs its nuclear localization, and therefore its transcriptional activity (39), suggesting that there is a potential field for developing drugs to modulate DAX-1 subcellular localization and consequently its function.