Ion channels and epilepsy in man and mouse

Inherited disorders of voltage-gated ion channels are a recently recognized etiology of epilepsy in the developing and mature central nervous system. Two human epilepsy syndromes, benign familial neonatal convulsions and gene ralized epilepsy with febrile seizures plus, represent K+ and Na+ channelop athies, and other newly defined syndromes have now been mapped to chromosom al regions that are rich in ion channel genes. Experimental mouse models pr omise a resolution of their intriguing pathophysiology, which includes a di verse array of cellular phenotypes consistent with the differential contrib utions of individual channels to excitability in neural networks.