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Heterogeneity of ciliary morphology in the immotile cilia syndrome in man



Heterogeneity of ciliary morphology in the immotile cilia syndrome in man



Journal of Ultrastructure Research 73(1): 34-43



Detailed ultrastructural analysis of the respiratory cilia of 3 men with the immotile cilia syndrome is presented. All had similar clinical manifestations, i.e., situs inversus totalis, repeated respiratory infections and sinusitis. Respiratory cilia of these patients showed a variety of structural abnormalities, some of which are described for the 1st time. In the cilia of the 1st patient both the dynein arms were lacking and almost 40% of the cilia had 1-9 supernumerary microtubules peripheral to the 9 outer doublets. The cilia of the 2nd patient showed an absence of inner dynein arms, unusually prominent outer dynein arms, a spoke defect in 46% of cilia examined and no morphologically detectable nexin links. The cilia of the last patient lacked both dynein arms but showed virtually no microtubular abnormalities. A variety of structural abnormalities in respiratory cilia can result in virtually identical clinical manifestations.

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Accession: 005565745

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PMID: 6450841


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