{"doi":"10.1203/pdr.0b013e318170a6b5","title":"Expression of Hepatocyte Transporters and Nuclear Receptors in Children With Early and Late-Stage Biliary Atresia","abstract":null,"journal":"Pediatric Research","year":2008,"id":612776,"datarank":0.6766289259775276,"base_score":4.51085950651685,"endowment":4.51085950651685,"self_citation_contribution":0.6766289259775276,"citation_network_contribution":0.0,"self_endowment_contribution":0.6766289259775276,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":90,"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":1577921,"name":"Yu-Jung Liu","orcid":null,"position":1,"is_corresponding":false},{"id":1510955,"name":"Hui-Ling Chen","orcid":null,"position":2,"is_corresponding":false},{"id":1577922,"name":"Shang-Hsin Wu","orcid":null,"position":3,"is_corresponding":false},{"id":1577923,"name":"Yen-Hsuan Ni","orcid":null,"position":4,"is_corresponding":false},{"id":1577924,"name":"Ming-Chih Ho","orcid":null,"position":5,"is_corresponding":false},{"id":1577925,"name":"Hong-Shiee Lai","orcid":null,"position":6,"is_corresponding":false},{"id":1577926,"name":"Wen-Ming Hsu","orcid":null,"position":7,"is_corresponding":false},{"id":1577927,"name":"Hong-Yuan Hsu","orcid":null,"position":8,"is_corresponding":false},{"id":1577928,"name":"Hui-Chih Tseng","orcid":null,"position":9,"is_corresponding":false},{"id":1577929,"name":"Yung-Ming Jeng","orcid":null,"position":10,"is_corresponding":false},{"id":1577930,"name":"Mei-Hwei Chang","orcid":"0000-0002-3648-9261","position":11,"is_corresponding":false},{"id":1577920,"name":"Huey-Ling Chen","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Expression of Hepatocyte Transporters and Nuclear Receptors in Children With Early and Late-Stage Biliary Atresia","abstract":"To investigate how the liver adapts to chronic obstructive cholestasis, liver samples from infants with early- and late-stage cholestasis were analyzed for changes in the levels of hepatocyte transporters and nuclear receptors. At early-stage cholestasis, most canalicular transporters and sinusoidal uptake transporters were downregulated, including bile salt export pump (BSEP, ABCB11), multidrug resistant protein 3 (MDR3, ABCB4), multidrug-resistant associated protein 2 (MRP2, ABCC2), sodium-dependent taurocholate cotransporting polypeptide (NTCP, SLC10A1), organic anion transporter (OATP, SLCO1A2), and nuclear receptor farnesoid X receptor (FXR, NR1H4). At late-stage cholestasis, FXR-BSEP levels returned to normal, MDR3 and MDR1 (ABCB1) were upregulated, and MRP-2 was downregulated. In addition, alternative sinusoidal efflux transporters, organic solute transporter alpha/beta (OSTalpha/beta) and MRP4 were upregulated, and pregnane X receptor (PXR, NR1I2) levels decreased. Cytochrome enzyme P450 7A1 was markedly downregulated at both early and late-stage cholestasis. An analysis of the long-term prognosis of 18 patients revealed lower PXR and constitutive androstane receptor (CAR, NR1I3) levels in the poor prognosis group. In conclusion, at long-term cholestasis, hepatocyte bile efflux was through sinusoidal and canalicular transporters, with FXR-BSEP levels maintained and PXR downregulated. Low PXR and CAR levels were associated with poor prognosis.","is_dataset_classified":null,"base_score":4.51085950651685,"endowment":4.51085950651685,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"18327154","pmcid":null,"openalex_id":"https://openalex.org/W2026624791","authors":[],"funders":[],"total_grants":0,"fwci":4.7775,"citation_percentile":0.94684007,"influential_citations":0,"citation_trend":[{"year":2012,"count":4},{"year":2013,"count":7},{"year":2014,"count":8},{"year":2015,"count":8},{"year":2016,"count":6},{"year":2017,"count":6},{"year":2018,"count":4},{"year":2019,"count":1},{"year":2020,"count":9},{"year":2021,"count":8},{"year":2022,"count":1},{"year":2023,"count":3},{"year":2024,"count":4},{"year":2025,"count":4},{"year":2026,"count":1}],"oa_status":"closed","license":null,"oa_locations":[{"url":"https://doi.org/10.1203/pdr.0b013e318170a6b5","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/18327154","host_type":"repository"}],"fields_of_study":["Pediatric Hepatobiliary Diseases and Treatments","Drug Transport and Resistance Mechanisms","Pancreatitis Pathology and Treatment","Adaptation, Physiological","Bile","Biliary Atresia","Cholestasis, Intrahepatic","Constitutive Androstane Receptor","Disease Progression","Female","Hepatocytes","Humans","Infant","Infant, Newborn","Liver","Liver Transplantation","Male","Membrane Transport Proteins","Microscopy, Fluorescence","Multidrug Resistance-Associated Protein 2","Prognosis","RNA, Messenger","Receptors, Cytoplasmic and Nuclear"],"mesh_terms":["Multidrug Resistance-Associated Protein 2","Constitutive Androstane Receptor","Adaptation, Physiological","Bile","Biliary Atresia","Cholestasis, Intrahepatic","Female","Humans","Infant","Infant, Newborn","Liver","Male","Microscopy, Fluorescence","Prognosis","RNA, Messenger","Liver Transplantation","Receptors, Cytoplasmic and Nuclear","Disease Progression","Hepatocytes","Membrane Transport Proteins"],"keywords":["Biliary atresia","Hepatocyte","Stage (stratigraphy)","Receptor","Transporter","Biology","Hepatocyte nuclear factors","Internal medicine","Medicine","Cell biology","Gene","Genetics","Gene expression"],"sdg_mappings":[{"sdg_number":0,"sdg_label":"No poverty"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-02T04:55:20.021984Z","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":[]}