Structure and function of neutrophil leukocytes from patients with the immotile-cilia syndrome

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. It was regarded of interest to examine whether other aspects of cell motility may also be involved. For this reason the functions and structure of the neutrophil leukocytes were examined. Cells from eight 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 E. 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 four of the eight patients. We conclude that the increased frequency of respiratory tract infections in patients with this syndrome is possibly due to defects in the granulocyte locomotory system, as well as to the defective mucociliary clearance of the airways.