de Santi M M, Gardi C, Barlocco G, Canciani M, Mastella G, Lungarella G
Istituti di Anatomia Patologica, Università di Siena, Italy.
Biol Cell. 1988;64(1):67-70. doi: 10.1016/0248-4900(88)90094-9.
This report describes the ultrastructural alterations observed in the nasal and bronchial mucosa of an 11-yr-old male suffering from immotile cilia syndrome (ICS). The morphological features observed in this patient are consistent with a ciliary aplasia. In fact, ciliated cells appeared to be replaced by columnar cells lacking cilia and basal bodies, and bearing on their surface cilium-like projections without any internal axonemal structure. In spite of the absence of basal bodies, centrioles, and kinocilia, these cells unexpectedly showed mature striated roots and centriolar precursor material scattered throughout the apical cytoplasm. These data suggest that control over basal body assembly is distinct from control over striated root formation. The presence of the above-reported structures in cells otherwise presenting many morphological features of normal ciliated cells is discussed on the basis of current knowledge of respiratory cilia biogenesis.
本报告描述了一名11岁患有不动纤毛综合征（ICS）男性患者鼻和支气管黏膜中观察到的超微结构改变。在该患者中观察到的形态学特征与纤毛发育不全一致。事实上，纤毛细胞似乎被缺乏纤毛和基体的柱状细胞所取代，并且在其表面带有没有任何内部轴丝结构的纤毛样突起。尽管没有基体、中心粒和动纤毛，但这些细胞意外地显示出成熟的横纹根和散布在顶端细胞质中的中心粒前体物质。这些数据表明，对基体组装的控制与对横纹根形成的控制是不同的。根据目前关于呼吸道纤毛生物发生的知识，讨论了在其他方面呈现正常纤毛细胞许多形态学特征的细胞中上述结构的存在情况。
