Structure and Function of Neutrophil Leukocytes from Patients with the Immotile‐Cilia Syndrome

Abstract

The various clinical manifestations of the recently characterized immotile-cilia syndrome can be traced to one cause: a structural defect of the cilia making them immotile. Whether other aspects of cell motility may also be involved was studied. For this reason the functions and structure of the neutrophil leukocytes were examined. Cells from 8 patients with the immotile-cilia syndrome and healthy controls were investigated with regard to random and stimulated locomotion under agarose, orientation during migration, adherence, bactericidal capacity and chemoluminescence. Four patients showed abnormally short migration distances of the leading front neutrophils after stimulation with serum and/or an [Escherichia coli] bacterial factor (BF). Ascorbic acid did not restore the defective migration. Migrating neutrophils were significantly less oriented towards the serum-containing agarose well compared with the controls (P < 0.01). Adherence, bactericidal capacity for Staphylococcus aureus, chemoluminescence, random migration, and orientation during BF-induced migration were all normal. The number of microtubules in the pericentriolar region of the neutrophil granulocytes was unusually low in 4 of the 8 patients. The increased frequency of respiratory tract infections in patients with this syndrome is possibly due to defects in the granulocyte locomotory system and to the defective mucociliary clearance of the airways.