ACTIVATION OF NASAL CILIA IN IMMOTILE CILIA SYNDROME

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Abstract
Nasal biopsy specimens were obtained from 5 normal subjects and from 7 patients with immotile cilia syndrome. Of the latter, 3 had Kartagener''s syndrome, 1 had Kartagener''s forme fruste and 3 had bronchiectasis and sinusitis. An in vitro motility test was used to assess ciliary movement. Exogenous ATP and ATPase activated the immotile cilia to levels equal to or slightly greater than the spontaneous activity seen in normal subjects. Absence of dynein arms on ciliary peripheral microtubule doublets was a consistent finding in the patients'' specimens and apparently is the basic defect in this syndrome that is responsible for immotility and absence of mucociliary clearance.

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