Domaratskiĭ K E, Uvakina E V, Volkov I K, Onishchenko G E
Ontogenez. 2005 May-Jun;36(3):190-8.
An electron microscopic study of the ciliary epithelium of respiratory tracts was carried out in children (members of the same family) with Kartagener syndrome, which is a variant of ciliary dyskinesia. It was shown that in the case of both mobile cilia and ciliary dyskinesia in man, centrioles are formed during formation of the ciliary basal bodies predominantly de novo, involving deuterosomes. A wide spectrum of pathological changes was described in literature, such as the absence of dynein arms in the axoneme and disorganization of axoneme structure. In addition to these changes in the ciliary system, we found integration of several ciliary axonemes by the same plasma membrane, running of microtubules from the plasma membrane as bundles, different orientation of basal legs, etc.
对患有卡塔格内综合征（一种纤毛运动障碍变体）的儿童（来自同一家族）的呼吸道纤毛上皮进行了电子显微镜研究。结果表明，在人类存在活动纤毛和纤毛运动障碍的情况下，纤毛基体形成过程中的中心粒主要是重新形成的，涉及双心体。文献中描述了广泛的病理变化，如轴丝中动力蛋白臂的缺失和轴丝结构的紊乱。除了纤毛系统的这些变化外，我们还发现同一质膜整合了多条纤毛轴丝、微管以束状形式从质膜延伸、基脚方向不同等情况。
