Extract

Primary ciliary dyskinesia (PCD), the second most common genetic muco-obstructive lung disease, is caused by mutations in over 50 different genes that encode proteins that are critical for the structure and/or function of motile cilia expressed at the apical membrane of ciliated airway epithelial cells [1–5]. These PCD genes include genes encoding proteins of the axonemal structure, regulatory complexes, ciliary assembly or ciliary transport [2, 6], and their absence or dysfunction causes an inborn defect of mucociliary clearance of inhaled pathogens and irritants leading to impaired host defence of the lung [3, 4, 7].

中文翻译：

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有效清除粘膜纤毛细胞需要多少个功能性纤毛气道上皮细胞？

 提取

原发性纤毛运动障碍 （PCD） 是第二常见的遗传性粘膜阻塞性肺病，是由 50 多个不同基因的突变引起的，这些基因编码的蛋白质对纤毛气道上皮细胞顶膜上表达的运动纤毛的结构和/或功能至关重要 [1–5]。这些 PCD 基因包括编码轴丝结构蛋白、调节复合物、纤毛组装或纤毛转运蛋白的基因 [2， 6]，它们的缺失或功能障碍会导致粘膜纤毛清除吸入病原体和刺激物的先天性缺陷，从而导致宿主对肺的防御受损 [3， 4， 7]。