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10.15252/embj.201490805

http://scihub22266oqcxt.onion/10.15252/embj.201490805
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C4406653!4406653!25712475
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pmid25712475      EMBO+J 2015 ; 34 (8): 1078-89
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  • Cyclin O (Ccno) functions during deuterosome-mediated centriole amplification of multiciliated cells #MMPMID25712475
  • Funk MC; Bera AN; Menchen T; Kuales G; Thriene K; Lienkamp SS; Dengjel J; Omran H; Frank M; Arnold SJ
  • EMBO J 2015[Apr]; 34 (8): 1078-89 PMID25712475show ga
  • Mucociliary clearance and fluid transport along epithelial surfaces are carried out by multiciliated cells (MCCs). Recently, human mutations in Cyclin O (CCNO) were linked to severe airway disease. Here, we show that Ccno expression is restricted to MCCs 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 MCCs. Ccno-deficient MCCs 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 MCCs.
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