PMID: 11923559Mar 30, 2002Paper

Freeze fracture study of airway epithelium from patients with primary ciliary dyskinesia

Thorax
C H ThaiJ L Carson

Abstract

The airway cilia of patients with primary ciliary dyskinesia (PCD) exhibit several anomalies when studied by transmission electron microscopy, but little is known about the ultrastructural organisation of ciliary membranes in these patients. Freeze fracture replication of airway epithelium from patients with PCD provides a means of achieving high resolution views of cell membrane structure. Ciliary necklaces are a specialised structural feature of ciliary membranes thought to serve as a timing mechanism for ciliary beat, and their characterisation in the cilia of patients with PCD may contribute new insights into the pathophysiology of this syndrome. The nasal epithelium of three patients with PCD was freeze fractured and replicated with platinum and carbon shadowing. The resultant preparations were examined by transmission electron microscopy and the ciliary necklaces were compared with similar preparations of nasal biopsy specimens from normal healthy subjects. The ciliary necklaces of the three patients with PCD were normal with no overt differences from those of healthy individuals. The defective ciliary motility observed in patients with PCD does not appear to result from membrane dysfunction associated with overt disorgan...Continue Reading

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Citations

Mar 30, 2002·Thorax·C BruceP S Thomas
May 9, 2008·PLoS Biology·Mélanie BonhiversDerrick R Robinson
Mar 17, 2004·Vojnosanitetski pregled. Military-medical and pharmaceutical review·Goran PlavecSlobodan Aćimović
Feb 16, 2011·Journal of Pediatric Health Care : Official Publication of National Association of Pediatric Nurse Associates & Practitioners·Rosalynn Bravo

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