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Potassium channel dysfunction in human neuronal models of Angelman syndrome.
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- Author(s): Alfred Xuyang Sun; Qiang Yuan; Masahiro Fukuda; Weonjin Yu; Haidun Yan; Grace Gui Yin Lim; Mui Hoon Nai; D’Agostino, Giuseppe Alessandro; Hoang-Dai Tran; Yoko Itahana; Danlei Wang; Lokman, Hidayat; Itahana, Koji; Wai Lin Lim, Stephanie; Jiong Tang; Ya Yin Chang; Menglan Zhang; Cook, Stuart A.; Rackham, Owen J. L.; Chwee Teck Lim
- Source:
Science. 12/20/2019, Vol. 366 Issue 6472, p1486-1492. 7p. 4 Diagrams. - Source:
- Additional Information
- Subject Terms:
- Abstract: Disruptions in the ubiquitin protein ligase E3A (UBE3A) gene cause Angelman syndrome (AS). Whereas AS model mice have associated synaptic dysfunction and altered plasticity with abnormal behavior, whether similar or other mechanisms contribute to network hyperactivity and epilepsy susceptibility in AS patients remains unclear. Using human neurons and brain organoids, we demonstrate that UBE3A suppresses neuronal hyperexcitability via ubiquitin-mediated degradation of calciumand voltage-dependent big potassium (BK) channels. We provide evidence that augmented BK channel activity manifests as increased intrinsic excitability in individual neurons and subsequent network synchronization. BK antagonists normalized neuronal excitability in both human and mouse neurons and ameliorated seizure susceptibility in an AS mouse model. Our findings suggest that BK channelopathy underlies epilepsy in AS and support the use of human cells to model human developmental diseases. [ABSTRACT FROM AUTHOR]
- Abstract: Copyright of Science is the property of American Association for the Advancement of Science and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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