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与不动纤毛综合征中所见的相比,获得性纤毛缺陷。

Acquired ciliary defects compared to those seen in the immotile-cilia syndrome.

作者信息

Afzelius B A, Camner P, Mossberg B

出版信息

Eur J Respir Dis Suppl. 1983;127:5-10.

PMID:6578058
Abstract

An attempt is made to characterize injured cilia and ciliary mutants separately with particular emphasis on human conditions. The usefulness of electron microscopical investigations is stressed. An injury to a ciliated epithelium may very well be restricted to one site in the body and may appear at any stage of life. Typical ciliary mutants, such as are seen in the immotile-cilia syndrome, represent a generalized condition and will be found from the beginning of life. Some types of ciliary defects are relatively unspecific and found after various kinds of insult to the cilia also in the immotile-cilia syndrome; these are: compound cilia, supernumerary microtubules in the axoneme, and loss of cilia or of ciliated cells. Other kinds of ciliary defect seem to be typical of the ciliary mutants: absence of dynein arms, absence of spokes and central sheath, transposition of one of the central microtubules and short central microtubules.

摘要

本文试图分别对受损纤毛和纤毛突变体进行特征描述,特别强调人类的情况。着重指出了电子显微镜检查的实用性。纤毛上皮的损伤很可能局限于身体的一个部位,并且可能出现在生命的任何阶段。典型的纤毛突变体,如在不动纤毛综合征中所见,代表一种全身性疾病,从生命开始就会出现。某些类型的纤毛缺陷相对不具特异性,在各种对纤毛的损伤后也能在不动纤毛综合征中发现;这些缺陷包括:复合纤毛、轴丝中微管数量过多、纤毛或纤毛细胞缺失。其他类型的纤毛缺陷似乎是纤毛突变体所特有的:动力蛋白臂缺失、辐条和中央鞘缺失、中央微管之一移位以及中央微管短小。

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