期刊
MOLECULAR BIOLOGY OF THE CELL
卷 25, 期 19, 页码 2919-2933出版社
AMER SOC CELL BIOLOGY
DOI: 10.1091/mbc.E14-02-0735
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资金
- National Institutes of Health Grants [R01GM52022, R01HL107493, R01GM086196]
- Intramural Program of the National Institute of Diabetes and Digestive and Kidney Diseases [ZIA DK075042]
- Singapore Agency for Science, Technology and Research
- National Science Foundation Graduate Fellowship
- National Center for Research Resources [1S10OD01227601]
Defects in centrosome and cilium function are associated with phenotypically related syndromes called ciliopathies. Cby1, the mammalian orthologue of the Drosophila Chibby protein, localizes to mature centrioles, is important for ciliogenesis in multiciliated airway epithelia in mice, and antagonizes canonical Wnt signaling via direct regulation of beta-catenin. We report that deletion of the mouse Cby1 gene results in cystic kidneys, a phenotype common to ciliopathies, and that Cby1 facilitates the formation of primary cilia and ciliary recruitment of the Joubert syndrome protein Arl13b. Localization of Cby1 to the distal end of mature centrioles depends on the centriole protein Ofd1. Superresolution microscopy using both three-dimensional SIM and STED reveals that Cby1 localizes to an similar to 250-nm ring at the distal end of the mature centriole, in close proximity to Ofd1 and Ahi1, a component of the transition zone between centriole and cilium. The amount of centriole-localized Ahi1, but not Ofd1, is reduced in Cby1(-/-) cells. This suggests that Cby1 is required for efficient recruitment of Ahi1, providing a possible molecular mechanism for the ciliogenesis defect in Cby1(-/-) cells.
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