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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.","is_dataset_classified":null,"base_score":0.0,"endowment":0.0,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"27158779","pmcid":"PMC4978421","openalex_id":null,"authors":[],"funders":[{"funder_name":"Medical Research Council","grant_id":"MR/L017237/1","title":null},{"funder_name":"Biotechnology and Biological Sciences Research Council","grant_id":"BB/K010492/1","title":null},{"funder_name":"NIDDK NIH HHS","grant_id":"R01 DK068306","title":null},{"funder_name":"NIAMS NIH HHS","grant_id":"R01 AR062651","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"RC2 HL103010","title":null},{"funder_name":"NHGRI NIH HHS","grant_id":"UM1 HG006493","title":null},{"funder_name":"NHGRI NIH HHS","grant_id":"UM1 HG006504","title":null},{"funder_name":"NIGMS NIH HHS","grant_id":"F32 GM112495","title":null},{"funder_name":"NIAMS NIH HHS","grant_id":"R01 AR061485","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"UC2 HL102926","title":null},{"funder_name":"NIAMS NIH HHS","grant_id":"R01 AR066124","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"UC2 HL103010","title":null},{"funder_name":"NCATS NIH HHS","grant_id":"UL1 TR000124","title":null},{"funder_name":"NIGMS NIH HHS","grant_id":"R01 GM121565","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"RC2 HL102923","title":null},{"funder_name":"NIGMS NIH HHS","grant_id":"DP1 GM106408","title":null},{"funder_name":"NIGMS NIH HHS","grant_id":"R01 GM086627","title":null},{"funder_name":"NIGMS NIH HHS","grant_id":"R01 GM114276","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"RC2 HL102925","title":null},{"funder_name":"Telethon","grant_id":"TGM16S01","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"UC2 HL102923","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"UC2 HL102925","title":null},{"funder_name":"NIGMS NIH HHS","grant_id":"R01 GM104853","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"R01 HL117164","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"RC2 HL102924","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"RC2 HL102926","title":null},{"funder_name":"NHGRI NIH HHS","grant_id":"U54 HG006493","title":null},{"funder_name":"NHLBI NIH HHS","grant_id":"UC2 HL102924","title":null},{"funder_name":"Howard Hughes Medical Institute","grant_id":"","title":null}],"total_grants":29,"fwci":null,"citation_percentile":null,"influential_citations":0,"citation_trend":[],"oa_status":"green","license":"other-oa","oa_locations":[{"url":"https://kclpure.kcl.ac.uk/ws/files/132295412/Toriyama_Submission_43308_3_merged_1458750927.pdf","host_type":"repository"},{"url":"http://www.nature.com/articles/ng.3558.pdf","host_type":"publisher"},{"url":"http://www.nature.com/articles/ng.3558","host_type":"publisher"},{"url":"http://nrs.harvard.edu/urn-3:HUL.InstRepos:29626113","host_type":"repository"},{"url":"https://kclpure.kcl.ac.uk/portal/en/publications/25609eb6-780c-46c9-a4e7-4ca2a8827ea1","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/4978421","host_type":"repository"},{"url":"https://europepmc.org/articles/PMC4978421","host_type":"Europe_PMC"},{"url":"https://europepmc.org/articles/PMC4978421?pdf=render","host_type":"Europe_PMC"}],"fields_of_study":[],"mesh_terms":["Flagella","Animals","Humans","Mice","Proteins","Protein Binding","Protein Transport","Phenotype","Mutation","Ciliopathies"],"keywords":[],"sdg_mappings":[],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-07-30T04:58:31.202570Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}