Wilsman N J, Morrison W B, Farnum C E, Fox L E
Am Rev Respir Dis. 1987 Jan;135(1):137-43. doi: 10.1164/arrd.1987.135.1.137.
Using a procedure of membrane solubilization followed by tannic acid fixation-staining, we found abnormally constructed central and B microtubules in cilia from dogs with primary ciliary dyskinesia. We also found that a ciliary abnormality commonly associated with primary ciliary dyskinesia, "shortened outer dynein arms," actually represents the absence of a specific set of the 3 peripheral subunits that make up the outer dynein arms. We believe that this is the first time that these have been reported. Because the proximal subunits of the outer dynein arm are present and attached to the A microtubule, we argue that in these patients there is no structural abnormality of the A microtubule or the A-tubule attachment sites for outer and inner arms and radial spokes. Also, because a majority of cilia in these patients were motile, we believe that the data from this study support the concept that the outer dynein arms are not required for some motion but probably required for normal motility; however, the central microtubules, central microtubular complex, and probably the B tubules are essential for motility.
通过一种先进行膜溶解，再用单宁酸固定染色的方法，我们在患有原发性纤毛运动障碍的犬类的纤毛中发现了中央微管和B微管结构异常。我们还发现，一种通常与原发性纤毛运动障碍相关的纤毛异常——“外动力蛋白臂缩短”，实际上是构成外动力蛋白臂的一组特定的3个外周亚基缺失。我们认为这是首次报道这些情况。由于外动力蛋白臂的近端亚基存在并附着于A微管，我们认为在这些患者中，A微管或外、内臂及辐条的A管附着位点没有结构异常。此外，由于这些患者中的大多数纤毛具有运动能力，我们认为这项研究的数据支持这样一种观点，即外动力蛋白臂对于某些运动不是必需的，但可能是正常运动所必需的；然而，中央微管、中央微管复合体以及可能的B微管对于运动是必不可少的。
