Journal
CURRENT OPINION IN GENETICS & DEVELOPMENT
Volume 10, Issue 3, Pages 286-291Publisher
CURRENT BIOLOGY LTD
DOI: 10.1016/S0959-437X(00)00079-4
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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 generalized epilepsy with febrile seizures plus, represent K+ and Na+ channelopathies, and other newly defined syndromes have now been mapped to chromosomal regions that are rich in ion channel genes. Experimental mouse models promise a resolution of their intriguing pathophysiology, which includes a diverse array of cellular phenotypes consistent with the differential contributions of individual channels to excitability in neural networks.
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