CILIARY DISEASES

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Ciliary dyskinesias: primary ciliary dyskinesia in adults

Primary ciliary dyskinesia (PCD) is a genetic disorder of cilia structure and function, chronic infections of the respiratory tract, fertility problems and disorders of organ laterality. Establishing a definitive diagnosis can be challenging, requiring a compatible phenotype and detection of ciliary functional and ultra-structural defects, along with newer screening tools such as nasal nitric o...

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Ciliary action.

To those interested in diseases of the chest, ciliary action should be of absorbing interest, not only through its importance in the interpretation and treatment of inflammatory infections, but also from its intrinsic physiological fascination. It is surprising that physicians place so much stress on the value of cough and so little on that of ciliary action; the former is really a pathological...

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Effect of Cilia Beat Frequency on Muco-ciliary Clearance

Background: The airway surface liquid (ASL), which is a ï‌‚uid layer coating the interior epithelial surface of the bronchi and bronchiolesis, plays an important defensive role against foreign particles and chemicals entering lungs.Objective: Numerical investigation has been employed to solve two-layer model consisting of mucus layer as a viscoelastic fluid and periciliary liquid layer as a New...

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Mucociliary function, ciliary ultrastructure, and ciliary orientation in Young's syndrome.

BACKGROUND Mucociliary clearance is impaired in patients with Young's syndrome (obstructive azoospermia with recurrent sinobronchial disease), cystic fibrosis, and primary ciliary dyskinesia. No defect of cilia or mucus has been detected in Young's syndrome. METHODS Ciliary function and ultrastructure, including ciliary orientation, were studied quantitatively in 20 patients with Young's synd...

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Ciliary Phosphoinositide Regulates Ciliary Protein Trafficking in Drosophila.

Cilia are highly specialized antennae-like cellular organelles. Inositol polyphosphate 5-phosphatase E (INPP5E) converts PI(4,5)P2 into PI4P and is required for proper ciliary function. Although Inpp5e mutations are associated with ciliopathies in humans and mice, the precise molecular role INPP5E plays in cilia remains unclear. Here, we report that Drosophila INPP5E (dINPP5E) regulates ciliary...

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ژورنال

عنوان ژورنال: Pediatric Research

سال: 1985

ISSN: 0031-3998,1530-0447

DOI: 10.1203/00006450-198510000-00146