Transposition of Ciliary Microtubules

Abstract

SPECIFIC defects of cilia1 , 2 in the respiratory mucosa have been implicated in the pathogenesis of Kartagener's syndrome, a hereditary disease characterized by situs inversus, bronchiectasis, and sinusitis3 and occurring in siblings with sinobronchial disease but without transposition of the viscera. The term "immotile cilia syndrome"4 has been proposed for the hereditary disease characterized by ultrastructural abnormalities in the dynein arms1 , 5 and the radial-spoke linkages2 of respiratory cilia and sperm. As part of a survey of children with respiratory disease, we have investigated two siblings with a chronic sinobronchial syndrome. We observed a new and distinctive structural defect in the cilia . . .