Defective neutrophil motility in patients with primary ciliary dyskinesia

Publisher Website
Google Scholar
Abstract
Microtubules are important in the regulation of the motile functions of a variety of cells, including leukocytes, ciliated cells and spermatozoa. Polymorphonuclear leukocyte [PML] function was studied in 10 patients with primary ciliary dyskinesia [PCD], an inherited disorder of microtubules in sperm tails and cilia. Neutrophil chemotaxis in Boyden chambers was slightly reduced, but only 1 patient showed a migration below normal values. In vitro mobilization of PML into skin windows was also slightly decreased. In contrast, neutrophil polarization and orientation was normal. The bactericidal actiivty of neutrophils from patients with PCD was normal, while the ingestion of bacteria was decreased. The abnormalities of neutrophil function in patients with PCD are related to motility. The microtubule defect responsible for the abnormal pattern of ciliary beating is apparently a general abnormality also responsible for the depression of PML motility.