Article
Dnah9 mutant mice and organoid models recapitulate the clinical features of patients with PCD and provide an excellent platform for drug screening.
Cell death & disease - 21 Jun 2022
Zheng Rui, Yang Wenhao, Wen Yuting, Xie Liang, Shi Fang, Lu Danli, Luo Jiaxin, Li Yan, Zhang Rui, Chen Ting, Chen Lina, Xu Wenming, Liu Hanmin
Abstract excerpt
Primary cilia dyskinesia (PCD) is a rare genetic disease caused by ciliary structural or functional defects. It causes severe outcomes in patients, including recurrent upper and lower airway infections, progressive lung failure, and randomization of heterotaxy. To date, although 50 genes have been shown to be responsible for PCD, the etiology remains elusive. Meanwhile, owing to the lack of a model mimicking the...
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