ArticlePLoS genetics2017
Conditional knockout mice for the distal appendage protein CEP164 reveal its essential roles in airway multiciliated cell differentiation.
Article in PLoS genetics, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 46 papers.
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Who cites it
46 citing papers in PubMed, 68 citations in OpenAlex.
- Basal body docking failure triggers centriole clustering and elimination in mammalian spermatogenesis.EMBO reports · 2026Article
- Loss of primary cilia in late pituitary organogenesis does not cause endocrine dysfunction.Journal of anatomy · 2026Article
- DEUP1 functions as a scaffold for basal foot integrity and planar polarity in multiciliated cells.Nature communications · 2026Article
- Centriole Duplication, Maturation, and Transformation During Mammalian Spermatogenesis.Advances in experimental medicine and biology · 2026Review
- Loss of cilia drives centriole clustering and elimination during mammalian spermatogenesis.bioRxiv : the preprint server for biology · 2025Article
- Cilia at the crossroad: convergence of regulatory mechanisms to govern cilia dynamics during cell signaling and the cell cycle.Cell & bioscience · 2025Review
- Function of manchette and intra-manchette transport in spermatogenesis and male fertility.Cell communication and signaling : CCS · 2025Review
- ciBAR1 loss in mice causes laterality defects, pancreatic degeneration, and altered glucose tolerance.Life science alliance · 2025Article
- Developmental and tissue-specific roles of mammalian centrosomes.The FEBS journal · 2025Review
- Disruption of distal appendage protein CEP164 causes skeletal malformation in mice.Biochemical and biophysical research communications · 2024Article
- Distinct roles of centriole distal appendage proteins in ciliary assembly and disassembly.Cell communication and signaling : CCS · 2024Article
- Proliferation associated 2G4 is required for the ciliation of vertebrate motile cilia.Communications biology · 2024Article
- Distinct roles of Kif6 and Kif9 in mammalian ciliary trafficking and motility.The Journal of cell biology · 2024Article
- ARL13B controls male reproductive tract physiology through primary and Motile Cilia.Communications biology · 2024Article
- Multiciliated ependymal cells: an update on biology and pathology in the adult brain.Acta neuropathologica · 2024Review
- The ARPKD Protein DZIP1L Regulates Ciliary Protein Entry by Modulating the Architecture and Function of Ciliary Transition Fibers.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2024Article
- The Cby3/ciBAR1 complex positions the annulus along the sperm flagellum during spermiogenesis.The Journal of cell biology · 2024Article
- Article
- Biallelic variants in CEP164 cause a motile ciliopathy-like syndrome.Clinical genetics · 2023Article
- Article
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Authors and funding
11 authors at 3 institutions in 1 country.
Funding
Abstract
Multiciliated cells of the airways, brain ventricles, and female reproductive tract provide the motive force for mucociliary clearance, cerebrospinal fluid circulation, and ovum transport. Despite their clear importance to human biology and health, the molecular mechanisms underlying multiciliated cell differentiation are poorly understood. Prior studies implicate the distal appendage/transition fiber protein CEP164 as a central regulator of primary ciliogenesis; however, its role in multiciliogenesis remains unknown. In this study, we have generated a novel conditional mouse model that lacks CEP164 in multiciliated tissues and the testis. These mice show a profound loss of airway, ependymal, and oviduct multicilia and develop hydrocephalus and male infertility. Using primary cultures of tracheal multiciliated cells as a model system, we found that CEP164 is critical for multiciliogenesis, at least in part, via its regulation of small vesicle recruitment, ciliary vesicle formation, and basal body docking. In addition, CEP164 is necessary for the proper recruitment of another distal appendage/transition fiber protein Chibby1 (Cby1) and its binding partners FAM92A and FAM92B to the ciliary base in multiciliated cells. In contrast to primary ciliogenesis, CEP164 is dispensable for the recruitment of intraflagellar transport (IFT) components to multicilia. Finally, we provide evidence that CEP164 differentially controls the ciliary targeting of membrane-associated proteins, including the small GTPases Rab8, Rab11, and Arl13b, in multiciliated cells. Altogether, our studies unravel unique requirements for CEP164 in primary versus multiciliogenesis and suggest that CEP164 modulates the selective transport of membrane vesicles and their cargoes into the ciliary compartment in multiciliated cells. Furthermore, our mouse model provides a useful tool to gain physiological insight into diseases associated with defective multicilia.
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