Premium
Defective neutrophil motility in patients with primary ciliary dyskinesia
Author(s) -
VALERIUS NIELS HENRIK,
KNUDSEN BODIL BYGUM,
PEDERSEN MOGENS
Publication year - 1983
Publication title -
european journal of clinical investigation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.164
H-Index - 107
eISSN - 1365-2362
pISSN - 0014-2972
DOI - 10.1111/j.1365-2362.1983.tb00134.x
Subject(s) - primary ciliary dyskinesia , chemotaxis , motility , cilium , microtubule , biology , motile cilium , secretion , flagellum , microbiology and biotechnology , immunology , in vivo , medicine , endocrinology , receptor , bacteria , genetics , bronchiectasis , lung
. Microtubules are important in the regulation of the motile functions of a variety of cells, including leukocytes, ciliated cells and spermatozoa. Polymorphonuclear leukocyte function was studied in ten patients with primary ciliary dyskinesia, an inherited disorder of microtubules in sperm tails and cilia. Neutrophil chemotaxis in Boyden chambers was slightly reduced, but only one patient showed a migration below normal values. In vivo mobilization of polymorphonuclear leukocytes into skin windows was also slightly decreased. In contrast, neutrophil polarization and orientation was normal. The bactericidal activity of neutrophils from patients with primary ciliary dyskinesia was normal, while the ingestion of bacteria was decreased. The abnormalities of neutrophil function in patients with primary ciliary dyskinesia are related to motility. It is suggested that the microtubule defect responsible for the abnormal pattern of ciliary beating is a general abnormality also responsible for the depression of polymorphonuclear leukocyte motility.