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Cyclin O ( Ccno ) functions during deuterosome‐mediated centriole amplification of multiciliated cells
Author(s) -
Funk Maja C,
Bera Agata N,
Menchen Tabea,
Kuales Georg,
Thriene Kerstin,
Lienkamp Soeren S,
Dengjel Jörn,
Omran Heymut,
Frank Marcus,
Arnold Sebastian J
Publication year - 2015
Publication title -
the embo journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.484
H-Index - 392
eISSN - 1460-2075
pISSN - 0261-4189
DOI - 10.15252/embj.201490805
Subject(s) - cilium , biology , centriole , motile cilium , mucociliary clearance , microbiology and biotechnology , basal body , phenotype , genetics , gene , microtubule , flagellum , lung , medicine
Mucociliary clearance and fluid transport along epithelial surfaces are carried out by multiciliated cells ( MCC s). Recently, human mutations in Cyclin O ( CCNO ) were linked to severe airway disease. Here, we show that Ccno expression is restricted to MCC s and the genetic deletion of Ccno in mouse leads to reduced numbers of multiple motile cilia and characteristic phenotypes of MCC dysfunction including severe hydrocephalus and mucociliary clearance deficits. Reduced cilia numbers are caused by compromised generation of centrioles at deuterosomes, which serve as major amplification platform for centrioles in MCC s. Ccno ‐deficient MCC s fail to sufficiently generate deuterosomes, and only reduced numbers of fully functional centrioles that undergo maturation to ciliary basal bodies are formed. Collectively, this study implicates CCNO as first known regulator of deuterosome formation and function for the amplification of centrioles in MCC s.