Potassium channel dysfunction in human neuronal models of Angelman syndrome
| العنوان: | Potassium channel dysfunction in human neuronal models of Angelman syndrome |
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| المؤلفون: | Mui Hoon Nai, Hoang-Dai Tran, Huck-Hui Ng, Chwee Teck Lim, Kah-Leong Lim, Haidun Yan, Koji Itahana, Menglan Zhang, Stephanie Wai Lin Lim, Qiang Yuan, Yoko Itahana, Giuseppe D'Agostino, Yong-hui Jiang, Alfred Xuyang Sun, Eng-King Tan, Danlei Wang, Hidayat Lokman, Owen J. L. Rackham, Weonjin Yu, Stuart A. Cook, Grace Lim, Ya Yin Chang, Masahiro Fukuda, Hyunsoo Shawn Je, Jiong Tang |
| المصدر: | Science. 366:1486-1492 |
| بيانات النشر: | American Association for the Advancement of Science (AAAS), 2019. |
| سنة النشر: | 2019 |
| مصطلحات موضوعية: | Ubiquitin-Protein Ligases, Models, Neurological, Mice, Epilepsy, Calcium Channels, N-Type, Channelopathy, Ubiquitin, Seizures, Angelman syndrome, Potassium Channel Blockers, UBE3A, medicine, Animals, Humans, Neurons, Multidisciplinary, Ubiquitin-Protein Ligase E3A, Voltage-dependent calcium channel, biology, Ubiquitination, medicine.disease, Potassium channel, Organoids, biology.protein, Angelman Syndrome, Neuroscience |
| الوصف: | Hyperexcitable neurons in brain organoids Individuals with Angelman syndrome experience intellectual disability and seizures throughout their lives. In this condition, ubiquitin-mediated degradation of a key potassium channel is disrupted, allowing for the neuronal excitability and network synchronization that leads to seizure. Sun et al. used brain organoid technology to study what happens in human neurons with a mutation in a ubiquitin ligase that is implicated in Angelman syndrome. In these in vitro models and in a mouse model of Angelman syndrome, antagonists for the potassium channel normalized neuronal excitability. Science , this issue p. 1486 |
| تدمد: | 1095-9203 0036-8075 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b132f4bf8fce823af213c44600c4a1df https://doi.org/10.1126/science.aav5386 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....b132f4bf8fce823af213c44600c4a1df |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 10959203 00368075 |
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