{"id":"https://openalex.org/W2005113281","doi":"https://doi.org/10.2350/04-12-0135.1","title":"Extrahepatic Biliary Atresia Demonstrates Abnormal Persistence of HES1 Protein in Neonatal Biliary Epithelium: An Immunohistochemical Study","display_name":"Extrahepatic Biliary Atresia Demonstrates Abnormal Persistence of HES1 Protein in Neonatal Biliary Epithelium: An Immunohistochemical Study","publication_year":2006,"publication_date":"2006-03-01","ids":{"openalex":"https://openalex.org/W2005113281","doi":"https://doi.org/10.2350/04-12-0135.1","mag":"2005113281","pmid":"https://pubmed.ncbi.nlm.nih.gov/16822088"},"language":"en","primary_location":{"id":"doi:10.2350/04-12-0135.1","doi":"https://doi.org/10.2350/04-12-0135.1","is_oa":false,"landing_page_url":"https://doi.org/10.2350/04-12-0135.1","pdf_url":null,"source":{"id":"https://openalex.org/S125957187","display_name":"Pediatric and Developmental Pathology","issn_l":"1093-5266","issn":["1093-5266","1615-5742"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","dongbi-c","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-40","russia-white-list-2"],"host_organization":"https://openalex.org/P4310320017","host_organization_name":"SAGE Publishing","host_organization_lineage":["https://openalex.org/P4310320017"],"host_organization_lineage_names":["SAGE Publishing"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pediatric and Developmental Pathology","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","pubmed"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5032313369","display_name":"Dinesh Rakheja","orcid":"https://orcid.org/0000-0001-6888-7902","observed_orcids":["https://orcid.org/0000-0001-6888-7902"]},"institutions":[{"id":"https://openalex.org/I1297463080","display_name":"Children's Medical Center","ror":"https://ror.org/02ndk3y82","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1297463080"]},{"id":"https://openalex.org/I867280407","display_name":"The University of Texas Southwestern Medical Center","ror":"https://ror.org/05byvp690","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I867280407"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Dinesh Rakheja","raw_affiliation_strings":["Department of Pathology, Children's Medical Center and University of Texas Southwestern Medical Center, Dallas, TX, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology, Children's Medical Center and University of Texas Southwestern Medical Center, Dallas, TX, USA","institution_ids":["https://openalex.org/I1297463080","https://openalex.org/I867280407"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5048073439","display_name":"Anirban Maitra","orcid":"https://orcid.org/0000-0001-7923-9978","observed_orcids":["https://orcid.org/0000-0001-7923-9978","https://orcid.org/0000-0003-0298-9775"]},"institutions":[{"id":"https://openalex.org/I145311948","display_name":"Johns Hopkins University","ror":"https://ror.org/00za53h95","country_code":"US","type":"education","lineage":["https://openalex.org/I145311948"]},{"id":"https://openalex.org/I2799853436","display_name":"Johns Hopkins Medicine","ror":"https://ror.org/037zgn354","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I2799853436"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Anirban Maitra","raw_affiliation_strings":["Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA","institution_ids":["https://openalex.org/I145311948","https://openalex.org/I2799853436"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5069595062","display_name":"Payal Kapur","orcid":"https://orcid.org/0000-0002-4239-0495","observed_orcids":["https://orcid.org/0000-0002-4239-0495"]},"institutions":[{"id":"https://openalex.org/I867280407","display_name":"The University of Texas Southwestern Medical Center","ror":"https://ror.org/05byvp690","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I867280407"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Payal Kapur","raw_affiliation_strings":["Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology, University of Texas Southwestern Medical Center, Dallas, TX, USA","institution_ids":["https://openalex.org/I867280407"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5113571350","display_name":"Arthur G. Weinberg","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I1297463080","display_name":"Children's Medical Center","ror":"https://ror.org/02ndk3y82","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I1297463080"]},{"id":"https://openalex.org/I867280407","display_name":"The University of Texas Southwestern Medical Center","ror":"https://ror.org/05byvp690","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I867280407"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Arthur G. Weinberg","raw_affiliation_strings":["Department of Pathology, Children's Medical Center and University of Texas Southwestern Medical Center, Dallas, TX, USA"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology, Children's Medical Center and University of Texas Southwestern Medical Center, Dallas, TX, USA","institution_ids":["https://openalex.org/I1297463080","https://openalex.org/I867280407"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":4,"corresponding_author_ids":["https://openalex.org/A5113571350"],"corresponding_institution_ids":["https://openalex.org/I1297463080","https://openalex.org/I867280407"],"apc_list":{"value":3400,"currency":"USD","value_usd":3400},"apc_paid":null,"fwci":0.6228,"has_fulltext":false,"cited_by_count":3,"citation_normalized_percentile":{"value":0.72679081,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":89,"max":94},"biblio":{"volume":"9","issue":"2","first_page":"98","last_page":"102"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T12460","display_name":"Pediatric Hepatobiliary Diseases and Treatments","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T12460","display_name":"Pediatric Hepatobiliary Diseases and Treatments","score":1.0,"subfield":{"id":"https://openalex.org/subfields/2746","display_name":"Surgery"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10518","display_name":"Gallbladder and Bile Duct Disorders","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10966","display_name":"Liver physiology and pathology","score":0.0,"subfield":{"id":"https://openalex.org/subfields/2721","display_name":"Hepatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/extrahepatic-biliary-atresia","display_name":"extrahepatic biliary atresia","score":1.0},{"id":"https://openalex.org/keywords/hes1","display_name":"Hes1","score":0.9900000095367432},{"id":"https://openalex.org/keywords/notch-signaling","display_name":"Notch signaling","score":0.9179999828338623},{"id":"https://openalex.org/keywords/biliary-epithelium","display_name":"biliary epithelium","score":0.8899999856948853},{"id":"https://openalex.org/keywords/bhlh-transcription-factors","display_name":"bHLH transcription factors","score":0.7429999709129333},{"id":"https://openalex.org/keywords/conjugated-hyperbilirubinemia","display_name":"conjugated hyperbilirubinemia","score":0.8999999761581421},{"id":"https://openalex.org/keywords/newborns","display_name":"neonates","score":0.8159999847412109},{"id":"https://openalex.org/keywords/gestational-development","display_name":"gestational development","score":0.7559999823570251},{"id":"https://openalex.org/keywords/diagnostic-biomarkers","display_name":"diagnostic biomarkers","score":0.7229999899864197}],"concepts":[{"id":"https://openalex.org/C2780283210","wikidata":"https://www.wikidata.org/wiki/Q18027301","display_name":"HES1","level":4,"score":0.8922325968742371},{"id":"https://openalex.org/C2910364697","wikidata":"https://www.wikidata.org/wiki/Q659033","display_name":"Extrahepatic Biliary Atresia","level":5,"score":0.8409544229507446},{"id":"https://openalex.org/C2779243279","wikidata":"https://www.wikidata.org/wiki/Q659033","display_name":"Biliary atresia","level":4,"score":0.6512688398361206},{"id":"https://openalex.org/C204232928","wikidata":"https://www.wikidata.org/wiki/Q899285","display_name":"Immunohistochemistry","level":2,"score":0.5595130920410156},{"id":"https://openalex.org/C86803240","wikidata":"https://www.wikidata.org/wiki/Q420","display_name":"Biology","level":0,"score":0.5343208909034729},{"id":"https://openalex.org/C2781149206","wikidata":"https://www.wikidata.org/wiki/Q757722","display_name":"Atresia","level":2,"score":0.4836234152317047},{"id":"https://openalex.org/C161879069","wikidata":"https://www.wikidata.org/wiki/Q904082","display_name":"Notch signaling pathway","level":3,"score":0.4451587498188019},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.44009724259376526},{"id":"https://openalex.org/C2775982439","wikidata":"https://www.wikidata.org/wiki/Q3562150","display_name":"Biliary tract","level":2,"score":0.4128470718860626},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.3644848167896271},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.3254866600036621},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.2869196832180023},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.2609274387359619},{"id":"https://openalex.org/C62478195","wikidata":"https://www.wikidata.org/wiki/Q828130","display_name":"Signal transduction","level":2,"score":0.2415582239627838},{"id":"https://openalex.org/C105702510","wikidata":"https://www.wikidata.org/wiki/Q514","display_name":"Anatomy","level":1,"score":0.17037421464920044},{"id":"https://openalex.org/C95444343","wikidata":"https://www.wikidata.org/wiki/Q7141","display_name":"Cell biology","level":1,"score":0.1698632538318634},{"id":"https://openalex.org/C2911091166","wikidata":"https://www.wikidata.org/wiki/Q106419912","display_name":"Transplantation","level":2,"score":0.0},{"id":"https://openalex.org/C2779609443","wikidata":"https://www.wikidata.org/wiki/Q1368191","display_name":"Liver transplantation","level":3,"score":0.0}],"mesh":[{"descriptor_ui":"D000072056","descriptor_name":"Transcription Factor HES-1","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000072056","descriptor_name":"Transcription Factor HES-1","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000072056","descriptor_name":"Transcription Factor HES-1","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000293","descriptor_name":"Adolescent","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000293","descriptor_name":"Adolescent","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000293","descriptor_name":"Adolescent","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000328","descriptor_name":"Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000328","descriptor_name":"Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000328","descriptor_name":"Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D001656","descriptor_name":"Biliary Atresia","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D001656","descriptor_name":"Biliary Atresia","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D001656","descriptor_name":"Biliary Atresia","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D001656","descriptor_name":"Biliary Atresia","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D001656","descriptor_name":"Biliary Atresia","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D001656","descriptor_name":"Biliary Atresia","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D002648","descriptor_name":"Child","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002648","descriptor_name":"Child","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002648","descriptor_name":"Child","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002675","descriptor_name":"Child, Preschool","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002675","descriptor_name":"Child, Preschool","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002675","descriptor_name":"Child, Preschool","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004848","descriptor_name":"Epithelium","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D004848","descriptor_name":"Epithelium","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D004848","descriptor_name":"Epithelium","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D004848","descriptor_name":"Epithelium","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D004848","descriptor_name":"Epithelium","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D004848","descriptor_name":"Epithelium","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007150","descriptor_name":"Immunohistochemistry","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007150","descriptor_name":"Immunohistochemistry","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007150","descriptor_name":"Immunohistochemistry","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007223","descriptor_name":"Infant","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007223","descriptor_name":"Infant","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007223","descriptor_name":"Infant","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007231","descriptor_name":"Infant, Newborn","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007231","descriptor_name":"Infant, Newborn","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007231","descriptor_name":"Infant, Newborn","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D015415","descriptor_name":"Biomarkers","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D015415","descriptor_name":"Biomarkers","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D015415","descriptor_name":"Biomarkers","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D017734","descriptor_name":"Bile Ducts, Extrahepatic","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D017734","descriptor_name":"Bile Ducts, Extrahepatic","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D017734","descriptor_name":"Bile Ducts, Extrahepatic","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D017734","descriptor_name":"Bile Ducts, Extrahepatic","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D017734","descriptor_name":"Bile Ducts, Extrahepatic","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D017734","descriptor_name":"Bile Ducts, Extrahepatic","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D018398","descriptor_name":"Homeodomain Proteins","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D018398","descriptor_name":"Homeodomain Proteins","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D018398","descriptor_name":"Homeodomain Proteins","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D051792","descriptor_name":"Basic Helix-Loop-Helix Proteins","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D051792","descriptor_name":"Basic Helix-Loop-Helix Transcription Factors","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D051792","descriptor_name":"Basic Helix-Loop-Helix Transcription Factors","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D051880","descriptor_name":"Receptors, Notch","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D051880","descriptor_name":"Receptors, Notch","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D051880","descriptor_name":"Receptors, Notch","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false}],"locations_count":2,"locations":[{"id":"doi:10.2350/04-12-0135.1","doi":"https://doi.org/10.2350/04-12-0135.1","is_oa":false,"landing_page_url":"https://doi.org/10.2350/04-12-0135.1","pdf_url":null,"source":{"id":"https://openalex.org/S125957187","display_name":"Pediatric and Developmental Pathology","issn_l":"1093-5266","issn":["1093-5266","1615-5742"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["cwts-core","dongbi-c","doyens","jufo-1","ki-jl-1","medline","norway-1","poland-40","russia-white-list-2"],"host_organization":"https://openalex.org/P4310320017","host_organization_name":"SAGE Publishing","host_organization_lineage":["https://openalex.org/P4310320017"],"host_organization_lineage_names":["SAGE Publishing"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pediatric and Developmental Pathology","raw_type":"journal-article"},{"id":"pmid:16822088","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/16822088","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society","raw_type":"Journal Article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.9476000070571899}],"sustainable_development_goals_aurora":[{"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being","score":0.8700000047683716}],"study_designs":[{"id":"https://openalex.org/study-designs/observational-study","display_name":"Observational Study"}],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":10,"referenced_works":["https://openalex.org/W1974185766","https://openalex.org/W2006640848","https://openalex.org/W2018042402","https://openalex.org/W2025748388","https://openalex.org/W2058734213","https://openalex.org/W2068894050","https://openalex.org/W2069529346","https://openalex.org/W2148054025","https://openalex.org/W4211170457","https://openalex.org/W4252869716"],"related_works":["https://openalex.org/W2007898372","https://openalex.org/W2073929498","https://openalex.org/W2237293534","https://openalex.org/W2072966096","https://openalex.org/W1983527388","https://openalex.org/W1989415637","https://openalex.org/W2158918896","https://openalex.org/W2025187874","https://openalex.org/W2332160328","https://openalex.org/W2766565931"],"abstract_inverted_index":{"Extrahepatic":[0],"biliary":[1,71,106,184],"atresia":[2],"(EHBA)":[3],"is":[4,14,37,79,98,131],"an":[5],"important":[6],"cause":[7],"of":[8,34,67,76,95,104,113,134,142,175],"conjugated":[9],"hyperbilirubinemia":[10],"in":[11,53,65,81,84,101,117,121,128,151,182],"neonates.":[12],"It":[13],"a":[15,19,51,92,155,161,179],"progressive":[16],"disease":[17],"with":[18],"poor":[20],"prognosis,":[21],"requiring":[22],"early":[23],"surgical":[24],"intervention":[25],"to":[26,49,110,139,160,171],"control":[27],"morbidity":[28],"and":[29,62,69],"mortality.":[30],"The":[31,56,73],"exact":[32],"pathogenesis":[33],"this":[35,96,135],"disorder":[36],"not":[38,116],"known,":[39],"although":[40],"genetic,":[41],"infectious,":[42],"toxic,":[43],"and/or":[44],"environmental":[45],"factors":[46],"are":[47],"thought":[48],"play":[50],"role":[52,174],"the":[54,85,102,122,126,173],"causation.":[55],"Notch":[57,86],"signaling":[58,83],"pathway":[59],"plays":[60],"diverse":[61],"critical":[63],"roles":[64],"development":[66],"extrahepatic":[68,183],"intrahepatic":[70],"tree.":[72],"HES":[74],"family":[75],"bHLH":[77],"proteins":[78],"involved":[80],"downstream":[82,163],"pathway.":[87],"We":[88,145],"demonstrate":[89],"that":[90,147],"HES1,":[91],"principal":[93],"member":[94],"family,":[97],"normally":[99],"expressed":[100],"nuclei":[103],"human":[105],"epithelial":[107],"cells":[108],"up":[109,138],"16":[111],"weeks":[112],"gestation,":[114],"but":[115],"later":[118],"gestation":[119],"or":[120],"neonatal":[123],"period.":[124],"On":[125],"contrary,":[127],"EHBA,":[129],"there":[130],"anomalous":[132],"persistence":[133],"protein":[136],"for":[137],"3":[140],"months":[141],"postnatal":[143],"life.":[144],"suggest":[146],"aberrant":[148],"HES1":[149,176],"expression":[150],"EHBA":[152],"may":[153],"represent":[154],"compensatory":[156],"feedback":[157],"upregulation":[158],"due":[159],"putative":[162],"molecular":[164],"defect.":[165],"Further":[166],"studies":[167],"should":[168],"be":[169],"performed":[170],"evaluate":[172],"immunohistochemistry":[177],"as":[178],"diagnostic":[180],"tool":[181],"atresia.":[185]},"counts_by_year":[{"year":2017,"cited_by_count":1}],"updated_date":"2026-10-21T09:56:26.102985","created_date":"2025-10-10T00:00:00"}
