دورية أكاديمية
Vestibular hair cells are more prone to damage by excessive acceleration insult in the mouse with KCNQ4 dysfunction
العنوان: | Vestibular hair cells are more prone to damage by excessive acceleration insult in the mouse with KCNQ4 dysfunction |
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المؤلفون: | Hansol Hong, Eun Ji Koo, Yesai Park, Gabae Song, Sun Young Joo, Jung Ah Kim, Heon Yung Gee, Jinsei Jung, Kangyoon Park, Gyu Cheol Han, Jae Young Choie, Sung Huhn Kim |
المصدر: | Scientific Reports, Vol 14, Iss 1, Pp 1-17 (2024) |
بيانات النشر: | Nature Portfolio, 2024. |
سنة النشر: | 2024 |
المجموعة: | LCC:Medicine LCC:Science |
مصطلحات موضوعية: | Medicine, Science |
الوصف: | Abstract KCNQ4 is a voltage-gated K+ channel was reported to distribute over the basolateral surface of type 1 vestibular hair cell and/or inner surface of calyx and heminode of the vestibular nerve connected to the type 1 vestibular hair cells of the inner ear. However, the precise localization of KCNQ4 is still controversial and little is known about the vestibular phenotypes caused by KCNQ4 dysfunction or the specific role of KCNQ4 in the vestibular organs. To investigate the role of KCNQ4 in the vestibular organ, 6-g hypergravity stimulation for 24 h, which represents excessive mechanical stimulation of the sensory epithelium, was applied to p.W277S Kcnq4 transgenic mice. KCNQ4 was detected on the inner surface of calyx of the vestibular afferent in transmission electron microscope images with immunogold labelling. Vestibular function decrease was more severe in the Kcnq4 p.W277S/p.W277S mice than in the Kcnq4 +/+ and Kcnq4 +/p.W277S mice after the stimulation. The vestibular function loss was resulted from the loss of type 1 vestibular hair cells, which was possibly caused by increased depolarization duration. Retigabine, a KCNQ activator, prevented hypergravity-induced vestibular dysfunction and hair cell loss. Patients with KCNQ4 mutations also showed abnormal clinical vestibular function tests. These findings suggest that KCNQ4 plays an essential role in calyx and afferent of type 1 vestibular hair cell preserving vestibular function against excessive mechanical stimulation. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 2045-2322 |
Relation: | https://doaj.org/toc/2045-2322 |
DOI: | 10.1038/s41598-024-66115-9 |
URL الوصول: | https://doaj.org/article/0a3b858434b44580b2f81fc3e9e32ae9 |
رقم الأكسشن: | edsdoj.0a3b858434b44580b2f81fc3e9e32ae9 |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 20452322 |
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DOI: | 10.1038/s41598-024-66115-9 |