Fig. 8: Drugs targeting mitochondrial could rescue the cilia beating and related phenotypes. | Cell Death & Disease

Fig. 8: Drugs targeting mitochondrial could rescue the cilia beating and related phenotypes.

From: Dnah9 mutant mice and organoid models recapitulate the clinical features of patients with PCD and provide an excellent platform for drug screening

Fig. 8

A Expression analysis of DNAH9, CCDC114, LLRC6, and GAS8 in ciliated epithelium digested from WT and Dnah9 KD mouse airway organoid. Green, ace-tubulin; red, DNAH9, LRRC6, CCDC114, and GAS8; blue, DAPI. Scale bar = 10 µm. B The quantified DNAH9, LRRC6, CCDC114, and GAS8 fluorescent intensities in cilia analyzed by ImageJ in (A). *P < 0.05; ns no significance, n = 3 per group. C The expression of immune factors in mouse airway organoid after 4-day poly (I:C) treatment. *P < 0.05; n = 3 per group. D Stepwise protocol for the organoid drug screen. E CBF of Dnah9 KD mouse airway organoid after 16-day drug treatment. *P < 0.05, n = 10 per group. F Western blot of TOMM20 and COX-IV in KD mouse airway organoid treated with ATP (10 µM, 50 µM) and NADH (10 µM, 25 µM, 50 µM) and WT organoid.

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