PMID: 15224888Jul 1, 2004Paper

The secondary nature of ciliary (dis)orientation in secondary and primary ciliary dyskinesia

Acta Oto-laryngologica
Mark Jorissen, Tom Willems

Abstract

Ciliary orientation (COR) is an important parameter of mucociliary clearance and ciliary disorientation has been reported in cases of acquired abnormalities [secondary ciliary dyskinesia (SCD)] and in a very few cases as the single abnormality in primary ciliary dyskinesia (PCD). The etiology, pathogenesis, consequences and relevance of ciliary (dis)orientation are still unclear. To elucidate the primary or secondary nature of ciliary (dis)orientation, COR was measured in 179 non-PCD and 59 PCD patients. COR was measured in biopsies and after ciliogenesis in culture and was correlated with a number of functional and ultrastructural parameters. COR was defined as the SD of the angles of lines through the central pair of microtubules using transmission electron microscopy. Internationally accepted normal values for COR are < or = 20 degrees; COR values of 20-35 degrees indicate increased disorientation; and COR values > 35 degrees represent a random orientation. For non-PCD biopsies, COR increased with increasing SCD, from 15 +/- 7 degrees (n = 54) for normal (< 5%) SCD to 28 +/- 8 degrees (n = 16) for severe (> 25%) SCD. No correlation was found between COR and ciliary beat frequency. However, increased COR values (28 +/- 8 degr...Continue Reading

References

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Citations

Apr 3, 2010·Nature Cell Biology·Wallace F Marshall
Mar 29, 2008·Journal of Veterinary Internal Medicine·K PalmersP Deprez
Nov 26, 2011·Diagnostic Pathology·Dirk Theegarten, Michael Ebsen
Jun 26, 2013·American Journal of Respiratory and Critical Care Medicine·Michael R KnowlesMargaret W Leigh
Jan 8, 2013·European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery·Ricardo Cassiano DemarcoWilma T Anselmo-Lima
Mar 24, 2006·Upsala Journal of Medical Sciences·Godfried M RoomansMarie Johannesson
Aug 16, 2016·Clinics in Chest Medicine·Michael R KnowlesMargaret Leigh
Sep 19, 2017·Ultrastructural Pathology·Mellisa Dixon, Amelia Shoemark
Feb 16, 2020·The European Respiratory Journal·Amelia ShoemarkUNKNOWN representing the BEAT-PCD Network Guideline Development Group
Dec 29, 2020·Journal of Structural Biology·Martin SchneiterStefan A Tschanz
May 29, 2021·Cell Communication and Signaling : CCS·Zuzanna Bukowy-Bieryłło
Nov 4, 2008·Archives of Pathology & Laboratory Medicine·Thomas P PlesecRichard A Prayson
Jan 16, 2008·Current Opinion in Cell Biology·Wallace F Marshall, Christopher Kintner

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