{"doi":"10.1007/s004410000215","title":"An ever-expanding story of cyst formation","abstract":null,"journal":"Cell and Tissue Research","year":2000,"id":674546,"datarank":0.3596842909197557,"base_score":2.3978952727983707,"endowment":2.3978952727983707,"self_citation_contribution":0.3596842909197557,"citation_network_contribution":0.0,"self_endowment_contribution":0.3596842909197557,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":10,"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":1762420,"name":"Nicholas Obermüller","orcid":null,"position":1,"is_corresponding":false},{"id":1762421,"name":"Anna Cedzich","orcid":null,"position":2,"is_corresponding":false},{"id":12849,"name":"Norbert Gretz","orcid":null,"position":3,"is_corresponding":false},{"id":1762422,"name":"Ralph Witzgall","orcid":null,"position":4,"is_corresponding":false},{"id":1762419,"name":"Anna Rachel Gallagher","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"An ever-expanding story of cyst formation","abstract":"Autosomal-dominant polycystic kidney disease represents one of the most common monogenetic human disorders. The cloning of the PKD1 and PKD2 genes, which are mutated in far more than 90% of the patients affected by this disease, has generated high hopes for a quick understanding of the pathogenesis of cyst formation. However, these expectations have not yet been fulfilled, since the function of both polycystin-1 and polycystin-2, the two proteins encoded by PKD1 and PKD2, still remains a puzzle. In this review, we will highlight some of the characteristics of polycystic kidney disease, briefly touch on polycystin-1, and then go on to describe recent results of experiments with polycystin-2, since the latter is the major focus of our work. We will discuss new evidence which suggests that autosomal-dominant polycystic kidney disease actually behaves recessively on a cellular level. Finally, a model will be presented that tries to explain the available data.","is_dataset_classified":null,"base_score":2.3978952727983707,"endowment":2.3978952727983707,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"10928266","pmcid":null,"openalex_id":"https://openalex.org/W2044425687","authors":[],"funders":[],"total_grants":0,"fwci":1.1929,"citation_percentile":0.7956499,"influential_citations":0,"citation_trend":[],"oa_status":"closed","license":"http://www.springer.com/tdm","oa_locations":[{"url":"http://link.springer.com/content/pdf/10.1007/s004410000215.pdf","host_type":"publisher"},{"url":"http://link.springer.com/article/10.1007/s004410000215/fulltext.html","host_type":"publisher"},{"url":"http://link.springer.com/content/pdf/10.1007/s004410000215","host_type":"publisher"},{"url":"https://doi.org/10.1007/s004410000215","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/10928266","host_type":"repository"}],"fields_of_study":["Genetic and Kidney Cyst Diseases","Renal and related cancers","Genetic Syndromes and Imprinting","Adrenal Glands","Animals","Endoplasmic Reticulum","Female","Glycosylation","Humans","Kidney","Membrane Proteins","Models, Biological","Ovary","Polycystic Kidney, Autosomal Dominant","Proteins","Rats","Salivary Glands","TRPP Cation Channels"],"mesh_terms":["Adrenal Glands","Animals","Endoplasmic Reticulum","Female","Glycosylation","Humans","Kidney","Membrane Proteins","Models, Biological","Ovary","Proteins","Salivary Glands","Polycystic Kidney, Autosomal Dominant","TRPP Cation Channels","Rats"],"keywords":["PKD1","Polycystic kidney disease","Disease","Polycystic kidney","Autosomal dominant polycystic kidney disease","Biology","Cyst","Pathogenesis","Cilium","Bioinformatics","Genetics","Kidney","Medicine","Computational biology","Pathology","Immunology"],"sdg_mappings":[{"sdg_number":0,"sdg_label":"Good health and well-being"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-16T17:16:10.751763Z","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":[]}