DC Field | Value | Language |
---|---|---|
dc.contributor.author | Oh, Hyoseon | ko |
dc.contributor.author | Lee, Suho | ko |
dc.contributor.author | Oh, Yusang | ko |
dc.contributor.author | Kim, Seongbin | ko |
dc.contributor.author | Kim, Young Seo | ko |
dc.contributor.author | Yang, Yeji | ko |
dc.contributor.author | Choi, Woochul | ko |
dc.contributor.author | Yoo, Ye-Eun | ko |
dc.contributor.author | Cho, Heejin | ko |
dc.contributor.author | Lee, Seungjoon | ko |
dc.contributor.author | Yang, Esther | ko |
dc.contributor.author | Koh, Wuhyun | ko |
dc.contributor.author | Won, Woojin | ko |
dc.contributor.author | Kim, Ryunhee | ko |
dc.contributor.author | Lee, C. Justin | ko |
dc.contributor.author | Kim, Hyun | ko |
dc.contributor.author | Kang, Hyojin | ko |
dc.contributor.author | Kim, Jin Young | ko |
dc.contributor.author | Ku, Taeyun | ko |
dc.contributor.author | Paik, Se-Bum | ko |
dc.contributor.author | Kim, Eunjoon | ko |
dc.date.accessioned | 2023-09-04T08:00:44Z | - |
dc.date.available | 2023-09-04T08:00:44Z | - |
dc.date.created | 2023-09-04 | - |
dc.date.created | 2023-09-04 | - |
dc.date.issued | 2023-06 | - |
dc.identifier.citation | NATURE COMMUNICATIONS, v.14, no.1 | - |
dc.identifier.issn | 2041-1723 | - |
dc.identifier.uri | http://hdl.handle.net/10203/312165 | - |
dc.description.abstract | Autism spectrum disorders (ASD) represent neurodevelopmental disorders characterized by social deficits, repetitive behaviors, and various comorbidities, including epilepsy. ANK2, which encodes a neuronal scaffolding protein, is frequently mutated in ASD, but its in vivo functions and disease-related mechanisms are largely unknown. Here, we report that mice with Ank2 knockout restricted to cortical and hippocampal excitatory neurons (Ank2-cKO mice) show ASD-related behavioral abnormalities and juvenile seizure-related death. Ank2-cKO cortical neurons show abnormally increased excitability and firing rate. These changes accompanied decreases in the total level and function of the Kv7.2/KCNQ2 and Kv7.3/KCNQ3 potassium channels and the density of these channels in the enlengthened axon initial segment. Importantly, the Kv7 agonist, retigabine, rescued neuronal excitability, juvenile seizure-related death, and hyperactivity in Ank2-cKO mice. These results suggest that Ank2 regulates neuronal excitability by regulating the length of and Kv7 density in the AIS and that Kv7 channelopathy is involved in Ank2-related brain dysfunctions. | - |
dc.language | English | - |
dc.publisher | NATURE PORTFOLIO | - |
dc.title | Kv7/KCNQ potassium channels in cortical hyperexcitability and juvenile seizure-related death in Ank2-mutant mice | - |
dc.type | Article | - |
dc.identifier.wosid | 001026275700013 | - |
dc.identifier.scopusid | 2-s2.0-85162066690 | - |
dc.type.rims | ART | - |
dc.citation.volume | 14 | - |
dc.citation.issue | 1 | - |
dc.citation.publicationname | NATURE COMMUNICATIONS | - |
dc.identifier.doi | 10.1038/s41467-023-39203-z | - |
dc.contributor.localauthor | Ku, Taeyun | - |
dc.contributor.localauthor | Paik, Se-Bum | - |
dc.contributor.localauthor | Kim, Eunjoon | - |
dc.contributor.nonIdAuthor | Lee, Suho | - |
dc.contributor.nonIdAuthor | Oh, Yusang | - |
dc.contributor.nonIdAuthor | Kim, Young Seo | - |
dc.contributor.nonIdAuthor | Yoo, Ye-Eun | - |
dc.contributor.nonIdAuthor | Cho, Heejin | - |
dc.contributor.nonIdAuthor | Lee, Seungjoon | - |
dc.contributor.nonIdAuthor | Yang, Esther | - |
dc.contributor.nonIdAuthor | Koh, Wuhyun | - |
dc.contributor.nonIdAuthor | Won, Woojin | - |
dc.contributor.nonIdAuthor | Kim, Ryunhee | - |
dc.contributor.nonIdAuthor | Lee, C. Justin | - |
dc.contributor.nonIdAuthor | Kim, Hyun | - |
dc.contributor.nonIdAuthor | Kang, Hyojin | - |
dc.contributor.nonIdAuthor | Kim, Jin Young | - |
dc.description.isOpenAccess | N | - |
dc.type.journalArticle | Article | - |
dc.subject.keywordPlus | AXON INITIAL SEGMENT | - |
dc.subject.keywordPlus | EPILEPSY-ASSOCIATED KCNQ2 | - |
dc.subject.keywordPlus | NEURONAL EXCITABILITY | - |
dc.subject.keywordPlus | MECHANISMS | - |
dc.subject.keywordPlus | EXPRESSION | - |
dc.subject.keywordPlus | ANKYRIN(B) | - |
dc.subject.keywordPlus | DISEASES | - |
dc.subject.keywordPlus | SODIUM | - |
dc.subject.keywordPlus | L1 | - |
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