{"doi":"10.1007/978-3-319-51436-9_11","title":"Primary Cilia in Cystic Kidney Disease","abstract":null,"journal":"Results and Problems in Cell Differentiation","year":2017,"id":668348,"datarank":0.5050943744979712,"base_score":3.367295829986474,"endowment":3.367295829986474,"self_citation_contribution":0.5050943744979712,"citation_network_contribution":0.0,"self_endowment_contribution":0.5050943744979712,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":28,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":499439,"name":"Robin L. Maser","orcid":"0000-0003-4737-0597","position":1,"is_corresponding":false},{"id":495418,"name":"Pamela V. Tran","orcid":"0000-0003-4619-4376","position":2,"is_corresponding":false},{"id":563672,"name":"Prachee Avasthi","orcid":"0000-0002-1688-722X","position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Primary Cilia in Cystic Kidney Disease","abstract":"Primary cilia are small, antenna-like structures that detect mechanical and chemical cues and transduce extracellular signals. While mammalian primary cilia were first reported in the late 1800s, scientific interest in these sensory organelles has burgeoned since the beginning of the twenty-first century with recognition that primary cilia are essential to human health. Among the most common clinical manifestations of ciliary dysfunction are renal cysts. The molecular mechanisms underlying renal cystogenesis are complex, involving multiple aberrant cellular processes and signaling pathways, while initiating molecular events remain undefined. Autosomal Dominant Polycystic Kidney Disease is the most common renal cystic disease, caused by disruption of polycystin-1 and polycystin-2 transmembrane proteins, which evidence suggests must localize to primary cilia for proper function. To understand how the absence of these proteins in primary cilia may be remediated, we review intracellular trafficking of polycystins to the primary cilium. We also examine the controversial mechanisms by which primary cilia transduce flow-mediated mechanical stress into intracellular calcium. Further, to better understand ciliary function in the kidney, we highlight the LKB1/AMPK, Wnt, and Hedgehog developmental signaling pathways mediated by primary cilia and misregulated in renal cystic disease.","is_dataset_classified":null,"base_score":3.367295829986474,"endowment":3.367295829986474,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"28409350","pmcid":"PMC7065811","openalex_id":"https://openalex.org/W2605644624","authors":[],"funders":[{"funder_name":"NIDDK NIH HHS","grant_id":"R01 DK103033","title":null}],"total_grants":1,"fwci":13.1469,"citation_percentile":0.98576349,"influential_citations":0,"citation_trend":[{"year":2018,"count":3},{"year":2019,"count":5},{"year":2020,"count":4},{"year":2021,"count":5},{"year":2023,"count":5},{"year":2024,"count":4},{"year":2025,"count":1},{"year":2026,"count":1}],"oa_status":"closed","license":"http://www.springer.com/tdm","oa_locations":[{"url":"https://link.springer.com/content/pdf/10.1007/978-3-319-51436-9_11","host_type":"publisher"},{"url":"https://doi.org/10.1007/978-3-319-51436-9_11","host_type":"book series"},{"url":"https://pubmed.ncbi.nlm.nih.gov/28409350","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/7065811","host_type":"repository"}],"fields_of_study":["Genetic and Kidney Cyst Diseases","Renal and related cancers","Hedgehog Signaling Pathway Studies","Animals","Cilia","Humans","Kidney Diseases, Cystic"],"mesh_terms":["Animals","Cilia","Humans","Kidney Diseases, Cystic"],"keywords":["Cilium","Polycystic kidney disease","Ciliogenesis","Biology","Cystic kidney disease","Intraflagellar transport","Primary ciliary dyskinesia","Cell biology","Nephronophthisis","Ciliopathies","Wnt signaling pathway","Ciliopathy","Bardet–Biedl syndrome","Signal transduction","Neuroscience","Kidney","Medicine","Internal medicine","Genetics","Flagellum","Gene"],"sdg_mappings":[{"sdg_number":0,"sdg_label":"Good health and well-being"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[{"name":"nct"}],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-13T21:08:41.677144Z","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":[]}