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Author

Park, Tae Joo
Developmental Morphogenesis Lab
Research Interests
  • Morphogenesis, chondrogenesis, ciliogenesis

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The ciliopathy-associated CPLANE proteins direct basal body recruitment of intraflagellar transport machinery

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Title
The ciliopathy-associated CPLANE proteins direct basal body recruitment of intraflagellar transport machinery
Author
Toriyama, MichinoriLee, ChanjaeTaypor, S PaigeDuran, IvanCohn, Daniel HBruel, Ange-LineTabler, Jacqueline MDrew, KevinKelly, Marcus RKim, SukyoungPark, Tae JooBraun, DaniellaPierquin, GhislaineBiver, ArmandWagner, KerstinMalfroot, AnnePanigrahi, InushaFranco, BrunellaAl-lami, Hadeel AdelYeung, YvonneChoi,Yeon JaUniversity WashingtonDuffourd,YannisFaivre, LaurenceRivière, Jean-BaptisteChen, JiangLiu, Karen JMarcotte, Edward MHildebrandt, FriedhelmThauvin-Robinet, CKrakow, DeborahJackson, Peter KWallingford, John B
Keywords
PLANAR CELL POLARITY; PRIMARY CILIARY DYSKINESIA; IFT-A COMPLEX; JOUBERT SYNDROME; CHLAMYDOMONAS FLAGELLA; EMBRYONIC-DEVELOPMENT; TUBULIN TRANSPORT; AXONEMAL DYNEINS; VESICLE DOCKING; EFFECTOR FUZ
Issue Date
201606
Publisher
NATURE PUBLISHING GROUP
Citation
NATURE GENETICS, v.48, no.6, pp.648 - 656
Abstract
Cilia use microtubule-based intraflagellar transport (IFT) to organize intercellular signaling. Ciliopathies are a spectrum of human diseases resulting from defects in cilia structure or function. The mechanisms regulating the assembly of ciliary multiprotein complexes and the transport of these complexes to the base of cilia remain largely unknown. Combining proteomics, in vivo imaging and genetic analysis of proteins linked to planar cell polarity (Inturned, Fuzzy and Wdpcp), we identified and characterized a new genetic module, which we term CPLANE (ciliogenesis and planar polarity effector), and an extensive associated protein network. CPLANE proteins physically and functionally interact with the poorly understood ciliopathy-associated protein Jbts17 at basal bodies, where they act to recruit a specific subset of IFT-A proteins. In the absence of CPLANE, defective IFT-A particles enter the axoneme and IFT-B trafficking is severely perturbed. Accordingly, mutation of CPLANE genes elicits specific ciliopathy phenotypes in mouse models and is associated with ciliopathies in human patients.
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DOI
http://dx.doi.org/10.1038/ng.3558
ISSN
1061-4036
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