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시냅스뇌질환연구단
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Kv7/KCNQ potassium channels in cortical hyperexcitability and juvenile seizure-related death in Ank2-mutant mice

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Title
Kv7/KCNQ potassium channels in cortical hyperexcitability and juvenile seizure-related death in Ank2-mutant mice
Author(s)
Hyoseon Oh; Suho Lee; Oh, Yusang; Kim, Seongbin; Kim, Young Seo; Yang, Yeji; Choi, Woochul; Ye-Eun Yoo; Heejin Cho; Seungjoon Lee; Yang, Esther; Wuhyun Koh; Woojin Won; Ryunhee Kim; C. Justin Lee; Kim, Hyun; Kang, Hyojin; Kim, Jin Young; Ku, Taeyun; Paik, Se-Bum; Eunjoon Kim
Publication Date
2023-06
Journal
Nature Communications, v.14, no.1, pp.3547
Publisher
Nature Research
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. © 2023, The Author(s).
URI
https://pr.ibs.re.kr/handle/8788114/13612
DOI
10.1038/s41467-023-39203-z
ISSN
2041-1723
Appears in Collections:
Center for Synaptic Brain Dysfunctions(시냅스 뇌질환 연구단) > 1. Journal Papers (저널논문)
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