{"doi":"10.1073/pnas.071516798","title":"Pendrin, encoded by the Pendred syndrome gene, resides in the apical region of renal intercalated cells and mediates bicarbonate secretion","abstract":"<jats:p>\n                    Pendrin is an anion transporter encoded by the\n                    <jats:italic>PDS</jats:italic>\n                    /\n                    <jats:italic>Pds</jats:italic>\n                    gene. In humans, mutations in\n                    <jats:italic>PDS</jats:italic>\n                    cause the genetic disorder Pendred syndrome, which\n is associated with deafness and goiter. Previous studies have shown\n that this gene has a relatively restricted pattern of expression, with\n                    <jats:italic>PDS</jats:italic>\n                    /\n                    <jats:italic>Pds</jats:italic>\n                    mRNA detected only in the\n thyroid, inner ear, and kidney. The present study examined the\n distribution and function of pendrin in the mammalian kidney.\n Immunolocalization studies were performed using anti-pendrin polyclonal\n and monoclonal antibodies. Labeling was detected on the apical surface\n of a subpopulation of cells within the cortical collecting ducts (CCDs)\n that also express the H\n                    <jats:sup>+</jats:sup>\n                    -ATPase but not aquaporin-2,\n indicating that pendrin is present in intercalated cells of the CCD.\n Furthermore, pendrin was detected exclusively within the subpopulation\n of intercalated cells that express the H\n                    <jats:sup>+</jats:sup>\n                    -ATPase but not\n the anion exchanger 1 (AE1) and that are thought to mediate bicarbonate\n secretion. The same distribution of pendrin was observed in mouse, rat,\n and human kidney. However, pendrin was not detected in kidneys from a\n                    <jats:italic>Pds</jats:italic>\n                    -knockout mouse. Perfused CCD tubules isolated from\n alkali-loaded wild-type mice secreted bicarbonate, whereas tubules from\n alkali-loaded\n                    <jats:italic>Pds</jats:italic>\n                    -knockout mice failed to secrete\n bicarbonate. Together, these studies indicate that pendrin is an apical\n anion transporter in intercalated cells of CCDs and has an essential\n role in renal bicarbonate secretion.\n                  </jats:p>","journal":"Proceedings of the National Academy of Sciences","year":2001,"id":643820,"datarank":0.9464877417339775,"base_score":6.309918278226516,"endowment":6.309918278226516,"self_citation_contribution":0.9464877417339775,"citation_network_contribution":0.0,"self_endowment_contribution":0.9464877417339775,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":549,"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":279523,"name":"Susan M. Wall","orcid":"0000-0003-2786-8849","position":1,"is_corresponding":false},{"id":1675392,"name":"Lawrence P. Karniski","orcid":null,"position":2,"is_corresponding":false},{"id":1675393,"name":"Lorraine A. Everett","orcid":null,"position":3,"is_corresponding":false},{"id":1516596,"name":"Koichi Suzuki","orcid":null,"position":4,"is_corresponding":false},{"id":264016,"name":"Mark A. Knepper","orcid":"0000-0002-2276-8091","position":5,"is_corresponding":false},{"id":19956,"name":"Eric D. Green","orcid":"0000-0002-2974-3823","position":6,"is_corresponding":false},{"id":1675391,"name":"Ines E. Royaux","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Pendrin, encoded by the Pendred syndrome gene, resides in the apical region of renal intercalated cells and mediates bicarbonate secretion","abstract":"<jats:p>\n                    Pendrin is an anion transporter encoded by the\n                    <jats:italic>PDS</jats:italic>\n                    /\n                    <jats:italic>Pds</jats:italic>\n                    gene. In humans, mutations in\n                    <jats:italic>PDS</jats:italic>\n                    cause the genetic disorder Pendred syndrome, which\n is associated with deafness and goiter. Previous studies have shown\n that this gene has a relatively restricted pattern of expression, with\n                    <jats:italic>PDS</jats:italic>\n                    /\n                    <jats:italic>Pds</jats:italic>\n                    mRNA detected only in the\n thyroid, inner ear, and kidney. The present study examined the\n distribution and function of pendrin in the mammalian kidney.\n Immunolocalization studies were performed using anti-pendrin polyclonal\n and monoclonal antibodies. Labeling was detected on the apical surface\n of a subpopulation of cells within the cortical collecting ducts (CCDs)\n that also express the H\n                    <jats:sup>+</jats:sup>\n                    -ATPase but not aquaporin-2,\n indicating that pendrin is present in intercalated cells of the CCD.\n Furthermore, pendrin was detected exclusively within the subpopulation\n of intercalated cells that express the H\n                    <jats:sup>+</jats:sup>\n                    -ATPase but not\n the anion exchanger 1 (AE1) and that are thought to mediate bicarbonate\n secretion. The same distribution of pendrin was observed in mouse, rat,\n and human kidney. However, pendrin was not detected in kidneys from a\n                    <jats:italic>Pds</jats:italic>\n                    -knockout mouse. Perfused CCD tubules isolated from\n alkali-loaded wild-type mice secreted bicarbonate, whereas tubules from\n alkali-loaded\n                    <jats:italic>Pds</jats:italic>\n                    -knockout mice failed to secrete\n bicarbonate. Together, these studies indicate that pendrin is an apical\n anion transporter in intercalated cells of CCDs and has an essential\n role in renal bicarbonate secretion.\n                  </jats:p>","is_dataset_classified":null,"base_score":6.309918278226516,"endowment":6.309918278226516,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"11274445","pmcid":"PMC31206","openalex_id":"https://openalex.org/W1988786406","authors":[],"funders":[{"funder_name":"NIDDK NIH HHS","grant_id":"R56 DK052935","title":null},{"funder_name":"Intramural NIH HHS","grant_id":"Z01 HL001285","title":null},{"funder_name":"Intramural NIH HHS","grant_id":"Z99 HL999999","title":null},{"funder_name":"NIDDK NIH HHS","grant_id":"DK52935","title":null},{"funder_name":"NIDDK NIH HHS","grant_id":"R01 DK052935","title":null}],"total_grants":5,"fwci":9.4021,"citation_percentile":0.98807683,"influential_citations":0,"citation_trend":[{"year":2012,"count":33},{"year":2013,"count":21},{"year":2014,"count":23},{"year":2015,"count":22},{"year":2016,"count":19},{"year":2017,"count":19},{"year":2018,"count":12},{"year":2019,"count":14},{"year":2020,"count":23},{"year":2021,"count":13},{"year":2022,"count":20},{"year":2023,"count":14},{"year":2024,"count":10},{"year":2025,"count":11},{"year":2026,"count":8}],"oa_status":"closed","license":null,"oa_locations":[{"url":"https://pnas.org/doi/pdf/10.1073/pnas.071516798","host_type":"publisher"},{"url":"https://doi.org/10.1073/pnas.071516798","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/11274445","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/31206","host_type":"repository"},{"url":"https://teikyo-u.repo.nii.ac.jp/records/2067005","host_type":"repository"}],"fields_of_study":["Ion channel regulation and function","Ion Transport and Channel Regulation","Hearing, Cochlea, Tinnitus, Genetics","Animals","Bicarbonates","Biological Transport","Carrier Proteins","Fluorescent Antibody Technique","Humans","In Vitro Techniques","Kidney","Kidney Tubules, Collecting","Membrane Transport Proteins","Mice","Molecular Sequence Data","RNA, Messenger","Rats","Sulfate Transporters","Tissue Distribution"],"mesh_terms":["Sulfate Transporters","Animals","Bicarbonates","Biological Transport","Carrier Proteins","Fluorescent Antibody Technique","Humans","Kidney","Kidney Tubules, Collecting","Molecular Sequence Data","RNA, Messenger","Tissue Distribution","Membrane Transport Proteins","Mice","Rats","In Vitro Techniques"],"keywords":["Pendrin","Apical membrane","Endocrinology","Bicarbonate","Secretion","Internal medicine","Biology","Chemistry","Molecular biology","Cell biology","Epithelium","Transporter","Biochemistry","Gene","Medicine","Genetics"],"sdg_mappings":[],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[{"name":"gen"}],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-08T19:06:27.219394Z","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":[]}