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Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2018,8,21]]},"abstract":"<jats:title>Significance<\/jats:title>\n                  <jats:p>\n                    Spider venom is a rich source of peptides, many targeting ion channels. We assessed a venom peptide, Hm1a, as a potential targeted therapy for Dravet syndrome, the genetic epilepsy linked to a mutation in the gene encoding the sodium channel alpha subunit Na\n                    <jats:sub>V<\/jats:sub>\n                    1.1. Cell-based assays showed Hm1a was selective for hNa\n                    <jats:sub>V<\/jats:sub>\n                    1.1 over other sodium and potassium channels. Utilizing a mouse model of Dravet syndrome, Hm1a restored inhibitory neuron function and significantly reduced seizures and mortality in heterozygote mice. Evidence from the structure of Hm1a and modeling suggest Hm1a interacts with Na\n                    <jats:sub>V<\/jats:sub>\n                    1.1 inactivation domains, providing a structural correlate of the functional mechanisms. This proof-of-concept study provides a promising strategy for future drug development in genetic epilepsy and other neurogenetic disorders.\n                  <\/jats:p>","DOI":"10.1073\/pnas.1804764115","type":"journal-article","created":{"date-parts":[[2018,8,3]],"date-time":"2018-08-03T15:39:26Z","timestamp":1533310766000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":134,"title":["Selective Na\n                    <sub>V<\/sub>\n                    1.1 activation rescues Dravet syndrome mice from seizures and premature death"],"prefix":"10.1073","volume":"115","author":[{"given":"Kay L.","family":"Richards","sequence":"first","affiliation":[{"name":"Ion Channels and Disease Group, Florey Institute of Neuroscience and Mental Health, Parkville, VIC 3010, Australia;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carol J.","family":"Milligan","sequence":"additional","affiliation":[{"name":"Ion Channels and Disease Group, Florey Institute of Neuroscience and Mental Health, Parkville, VIC 3010, Australia;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J.","family":"Richardson","sequence":"additional","affiliation":[{"name":"Ion Channels and Disease Group, Florey Institute of Neuroscience and Mental Health, Parkville, VIC 3010, Australia;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikola","family":"Jancovski","sequence":"additional","affiliation":[{"name":"Ion Channels and Disease Group, Florey Institute of Neuroscience and Mental Health, Parkville, VIC 3010, Australia;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Morten","family":"Grunnet","sequence":"additional","affiliation":[{"name":"Neuroscience Drug Discovery, H. 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