Characterization of a chemotactic defect in patients with Kartagener syndrome.
Kartagener syndrome (KS) is an autosomally inherited recessive condition characterized by situs inversus, bronchiectasis, and chronic sinusitis. Ciliary dynein, the mechanochemical force generator in ciliary movement, is deficient in patients with KS. We examined blood samples from two patients and tissue biopsy specimens from five patients and found: (1) no significant defect in neutrophil or monocyte chemotaxis in response to formylpeptide chemoattractant; (2) no alterations in centriolar structure, but significantly more centriole-associated microtubules in KS neutrophils and monocytes than in control leukocytes; and (3) a marked reduction in KS fibroblast chemotaxis in response to fibronectin compared with control fibroblasts. The significance of these cellular defects in KS is described.
Walter
R J
RJ
Department of Surgery, Cook County Hospital, Hektoen Institute for Medical Research, Chicago, Ill 60612.
Danielson
J R
JR
Reyes
H M
HM
D000251:Adenosine Triphosphatases / Q000172:deficiency*; D002478:Cells, Cultured; D002633:Chemotaxis / Q000502:physiology*; D002634:Chemotaxis, Leukocyte / Q000502:physiology*; D002923:Cilia / Q000378:metabolism; D003599:Cytoskeleton / Q000648:ultrastructure; D004398:Dyneins / Q000172:deficiency*; D005347:Fibroblasts / Q000502:physiology; D006801:Humans; D007619:Kartagener Syndrome / Q000235:genetics / Q000503:physiopathology*; D007963:Leukocytes, Mononuclear / Q000502:physiology / Q000648:ultrastructure; D008854:Microscopy, Electron; D008870:Microtubules / Q000648:ultrastructure; D009504:Neutrophils / Q000502:physiology / Q000648:ultrastructure
D000251:Adenosine Triphosphatases; D004398:Dyneins