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Dev Biol. 2019 Dec 27. pii: S0012-1606(19)30471-3. doi: 10.1016/j.ydbio.2019.12.010. [Epub ahead of print]

CFAP43 modulates ciliary beating in mouse and Xenopus.

Author information

1
Institute for Molecular Biology, OE5250, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
2
Institute of Zoology, University of Hohenheim, Garbenstraße 30, 70593, Stuttgart, Germany.
3
Institute of Functional and Applied Anatomy, Research Core Unit Electron Microscopy, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany.
4
Institute of Ophthalmic Research, Center for Ophthalmology, University of Tübingen, Röntgenweg 11, 72076, Tübingen, Germany.
5
Institute of Molecular Immunology, Helmholtz Zentrum München, German Research Center for Environmental Health (GmbH), Core Facility Monoclonal Antibodies, Marchioninistr. 25, 81377, München, Germany.
6
Institute of Zoology, University of Hohenheim, Garbenstraße 30, 70593, Stuttgart, Germany. Electronic address: martin.blum@uni-hohenheim.de.
7
Institute for Molecular Biology, OE5250, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany. Electronic address: gossler.achim@mh-hannover.de.

Abstract

Malfunctions of motile cilia cause a variety of developmental defects and diseases in humans and animal model organisms. Defects include impaired mucociliary clearance of the airways, sperm immotility, hydrocephalus and organ laterality. Here, we characterize the evolutionary conserved Cfap43 gene by loss-of-function experiments in the mouse and the frog Xenopus laevis. Cfap43 is expressed in tissues carrying motile cilia and acts as a target gene of the transcription factor FOXJ1, which is essential for the induction of motile ciliogenesis. We show that CFAP43, a protein of unknown biochemical function, localizes to the ciliary axoneme. CFAP43 is involved in the regulation of the beating frequency of tracheal cilia and loss of CFAP43 causes severe mucus accumulation in the nasal cavity. Likewise, morphant and crispant frog embryos revealed impaired function of motile cilia of the larval epidermis, a model for airway mucociliary epithelia. CFAP43 participates in the formation of flagellar axonemes during spermatogenesis as mice mutant for Cfap43 display male infertility, consistent with observations in male sterile patients. In addition, mice mutant for Cfap43 display early onset hydrocephalus. Together, these results confirm the role of CFAP43 in the male reproductive tract and pinpoint additional functions in airway epithelia mucus clearance and brain development.

KEYWORDS:

CFAP43; Cilia; Ciliogenesis; Hydrocephalus; Male infertility; Mouse; Mucociliary clearance; Xenopus laevis

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