Immotile cilia syndrome (primary ciliary dyskinesia) and inflammatory lung disease.
Abnormal mucociliary clearance can either be due to defects of mucus production and rheology or to abnormalities of the cilia. Ciliary dysfunction can be inherited as a genetically determined defect in axonemal structure (called the immotile cilia syndrome or primary ciliary dyskinesia) or acquired defects can result from airway injury. This article examines the defense of the lung as related to ciliary activity and addresses ways in which this activity can be modified by inflammatory mechanisms.
Rubin
B K
BK
Department of Pediatrics, University of Alberta, Edmonton, Canada.
D002923:Cilia / Q000648:ultrastructure; D002925:Ciliary Motility Disorders / Q000235:genetics / Q000473:pathology; D006801:Humans; D008854:Microscopy, Electron; D011014:Pneumonia / Q000473:pathology