Article (Scientific journals)
To beat, or not to beat, that is question! The spectrum of ciliopathies.
Kempeneers, Céline; Chilvers, Mark A.
2018In Pediatric Pulmonology, 53 (8), p. 1122-1129
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Keywords :
cilia; ciliopathie; motile cilia; non-motile cilia; primary ciliary dyskinesia; respiratory cilia
Abstract :
[en] Cilia are widely distributed throughout the human body, and have numerous roles in physiology, development, and disease. Ciliary ultrastructure is complex, consisting of nine parallel microtubules doublets, with or without motor dynein arms and a central pair of microtubules. Classification of cilia has evolved over time, and currently, four main classes are described: motile and non-motile cilia with a "9 + 2" structure, and motile and non-motile cilia with a "9 + 0" structure, which depend on the presence or absence of dynein arms and a central pair. Ciliopathies are inherited multisystem disorders of cilia, and may present with a varied spectrum of genotypes and phenotypes. Motor and sensory ciliopathies were historically considered as distinct dysfunctions of motile and non-motile cilia, but recent data indicate that the classical features of motor and sensory cilia may overlap.
Disciplines :
Pediatrics
Cardiovascular & respiratory systems
Author, co-author :
Kempeneers, Céline  ;  Université de Liège - ULiège > Département des sciences cliniques > Département des sciences cliniques
Chilvers, Mark A.;  University of British Columbia and British Columbia Children's Hospital, Vancouver, BC, Canada > Pediatrics > Respirology
Language :
English
Title :
To beat, or not to beat, that is question! The spectrum of ciliopathies.
Publication date :
25 August 2018
Journal title :
Pediatric Pulmonology
ISSN :
8755-6863
eISSN :
1099-0496
Publisher :
John Wiley & Sons, Hoboken, United States - New York
Volume :
53
Issue :
8
Pages :
1122-1129
Peer reviewed :
Peer Reviewed verified by ORBi
Commentary :
Epub ahead of print. (c) 2018 Wiley Periodicals, Inc.
Available on ORBi :
since 27 December 2018

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