Journal
ANNUAL REVIEW OF PHARMACOLOGY AND TOXICOLOGY, VOL 54
Volume 54, Issue -, Pages 317-338Publisher
ANNUAL REVIEWS
DOI: 10.1146/annurev-pharmtox-011112-140232
Keywords
sodium channels; structure; epilepsy; antiepileptic drugs; ion channelopathies
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Funding
- NINDS NIH HHS [R01 NS015751, R01 NS025704] Funding Source: Medline
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Voltage-gated sodium channels initiate action potentials in brain neurons, mutations in sodium channels cause inherited forms of epilepsy, and sodium channel blockers-along with other classes of drugs-are used in therapy of epilepsy. A mammalian voltage-gated sodium channel is a complex containing a large, pore-forming alpha subunit and one or two smaller beta subunits. Extensive structure-function studies have revealed many aspects of the molecular basis for sodium channel structure, and X-ray crystallography of ancestral bacterial sodium channels has given insight into their three-dimensional structure. Mutations in sodium channel alpha and beta subunits are responsible for genetic epilepsy syndromes with a wide range of severity, including generalized epilepsy with febrile seizures plus (GEFS+), Dravet syndrome, and benign familial neonatal-infantile seizures. These seizure syndromes are treated with antiepileptic drugs that offer differing degrees of success. The recent advances in understanding of disease mechanisms and sodium channel structure promise to yield improved therapeutic approaches.
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