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The FOXJ1 target Cfap206 is required for sperm motility, mucociliary clearance of the airways and brain development.
Development 147:dev188052 (2020)
Publ. Version/Full Text
DOI
PMC
Cilia are complex cellular protrusions consisting of hundreds of proteins. Defects in ciliary structure and function, many of which have not been characterised molecularly, cause ciliopathies: a heterogeneous group of human syndromes. Here, we report on the FOXJ1 target gene Cfap206, orthologues of which so far have only been studied in Chlamydomonas and Tetrahymena In mouse and Xenopus, Cfap206 was co-expressed with and dependent on Foxj1 CFAP206 protein localised to the basal body and to the axoneme of motile cilia. In Xenopus crispant larvae, the ciliary beat frequency of skin multiciliated cells was enhanced and bead transport across the epidermal mucociliary epithelium was reduced. Likewise, Cfap206 knockout mice revealed ciliary phenotypes. Electron tomography of immotile knockout mouse sperm flagella indicated a role in radial spoke formation reminiscent of FAP206 function in Tetrahymena Male infertility, hydrocephalus and impaired mucociliary clearance of the airways in the absence of laterality defects in Cfap206 mutant mice suggests that Cfap206 may represent a candidate for the subgroup of human primary ciliary dyskinesias caused by radial spoke defects.
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Publication type
Article: Journal article
Document type
Scientific Article
Keywords
Ciliary Beat Frequency ; Hydrocephalus ; Male Infertility ; Motile Cilia ; Mucus Accumulation ; Radial Spoke Defect; Primary Ciliary Dyskinesia; Computational Platform; Ependymal Cells; Ciliogenesis; Identification; Mutations; Flagellar; Protein; Flow; Hydrocephalus
ISSN (print) / ISBN
0950-1991
e-ISSN
1477-9129
Quellenangaben
Volume: 147,
Issue: 21,
Article Number: dev188052
Publisher
Company of Biologists
Publishing Place
Bidder Building, Station Rd, Histon, Cambridge Cb24 9lf, England
Non-patent literature
Publications
Reviewing status
Peer reviewed
Institute(s)
Institute of Molecular Immunology (IMI)
Grants
Landesgraduiertenforderung Baden-Wurttemberg
Deutsche Forschungsgemeinschaft Cluster of Excellence 'REBIRTH'
Deutsche Forschungsgemeinschaft
Deutsche Forschungsgemeinschaft Cluster of Excellence 'REBIRTH'
Deutsche Forschungsgemeinschaft