{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T02:56:06Z","timestamp":1791082566929,"version":"4.1.0"},"reference-count":27,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"6","license":[{"start":{"date-parts":[[2011,5,2]],"date-time":"2011-05-02T00:00:00Z","timestamp":1304294400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2011,6,1]],"date-time":"2011-06-01T00:00:00Z","timestamp":1306886400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2011,6]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>\n                                 <jats:bold>Ductal plate malformations (DPMs) are developmental anomalies considered to result from lack of ductal plate remodeling during bile duct morphogenesis. In mice, bile duct development is initiated by the formation of primitive ductal structures lined by two cell types, namely ductal plate cells and hepatoblasts. During ductal plate remodeling, the primitive ductal structures mature to ducts as a result from differentiation of the ductal plate cells and hepatoblasts to cholangiocytes. Here, we report this process is conserved in human fetal liver. These findings prompted us to evaluate how DPMs develop in three mouse models, namely mice with livers deficient in hepatocyte nuclear factor 6 (HNF6), HNF1\u03b2, or cystin-1 ( cpk [congenital polycystic kidney] mice). Human liver from a patient with a HNF1B\/TCF2 mutation, and from fetuses affected with autosomal recessive polycystic kidney disease (ARPKD) were also analyzed. Despite the epistatic relationship between HNF6, HNF1\u03b2, and cystin-1, the three mouse models displayed distinct morphogenic mechanisms of DPM. They all developed biliary cysts lined by cells with abnormal apicobasal polarity. However, the absence of HNF6 led to an early defect in ductal plate cell differentiation. In HNF1\u03b2-deficient liver, maturation of the primitive ductal structures was impaired. Normal differentiation and maturation but abnormal duct expansion was apparent in cpk mouse livers and in human fetal ARPKD.<\/jats:bold>\n                              <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusion:<\/jats:title>\n            <jats:p>\n                                 <jats:bold>DPM is the common endpoint of distinct defects initiated at distinct stages of bile duct morphogenesis. Our observations provide a new pathogenic classification of DPM. (HEPATOLOGY 2011;)<\/jats:bold>\n                              <\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.24292","type":"journal-article","created":{"date-parts":[[2011,3,9]],"date-time":"2011-03-09T17:58:39Z","timestamp":1299693519000},"page":"1959-1966","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":106,"title":["A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis"],"prefix":"10.1097","volume":"53","author":[{"given":"Peggy","family":"Raynaud","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joshua","family":"Tate","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9line","family":"Callens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Cordi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Vandersmissen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rodolphe","family":"Carpentier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christine","family":"Sempoux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Devuyst","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christophe E.","family":"Pierreux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Courtoy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karin","family":"Dahan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katty","family":"Delbecque","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S\u00e9bastien","family":"Lepreux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Pontoglio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa M.","family":"Guay-Woodford","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9d\u00e9ric P.","family":"Lemaigre","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-20-20241201","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1002\/hep.1840160434","article-title":"Congenital diseases of intrahepatic bile ducts: variations on the theme ductal plate malformation.","volume":"16","author":"Desmet","year":"1992","journal-title":"HEPATOLOGY"},{"key":"R2-20-20241201","doi-asserted-by":"crossref","first-page":"260","DOI":"10.1111\/j.1365-2559.2004.01945.x","article-title":"Morphological and immunohistochemical analysis of ductal plate malformation: correlation with fetal liver.","volume":"45","author":"Awasthi","year":"2004","journal-title":"Histopathology"},{"key":"R3-20-20241201","doi-asserted-by":"crossref","first-page":"2325","DOI":"10.1053\/j.gastro.2009.02.051","article-title":"Intrahepatic bile ducts develop according to a new mode of tubulogenesis regulated by the transcription factor SOX9.","volume":"136","author":"Antoniou","year":"2009","journal-title":"Gastroenterology"},{"key":"R4-20-20241201","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.biocel.2009.07.020","article-title":"Biliary differentiation and bile duct morphogenesis in development and disease.","volume":"43","author":"Raynaud","year":"2011","journal-title":"Int J Biochem Cell Biol"},{"key":"R5-20-20241201","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1002\/ar.20710","article-title":"Embryology of extra- and intrahepatic bile ducts, the ductal plate.","volume":"291","author":"Roskams","year":"2008","journal-title":"Anat Rec"},{"key":"R6-20-20241201","doi-asserted-by":"crossref","first-page":"1819","DOI":"10.1242\/dev.129.8.1819","article-title":"The onecut transcription factor HNF6 is required for normal development of the biliary tract.","volume":"129","author":"Clotman","year":"2002","journal-title":"Development"},{"key":"R7-20-20241201","doi-asserted-by":"crossref","first-page":"1829","DOI":"10.1242\/dev.129.8.1829","article-title":"Bile system morphogenesis defects and liver dysfunction upon targeted deletion of HNF1beta.","volume":"129","author":"Coffinier","year":"2002","journal-title":"Development"},{"key":"R8-20-20241201","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1053\/j.gastro.2005.12.005","article-title":"The transcription factor hepatocyte nuclear factor-6 controls the development of pancreatic ducts in the mouse.","volume":"130","author":"Pierreux","year":"2006","journal-title":"Gastroenterology"},{"key":"R9-20-20241201","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1038\/ng833","article-title":"The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein.","volume":"30","author":"Ward","year":"2002","journal-title":"Nat Genet"},{"key":"R10-20-20241201","doi-asserted-by":"crossref","first-page":"2246","DOI":"10.1097\/01.ASN.0000030392.19694.9D","article-title":"Identification and characterization of Pkhd1, the mouse orthologue of the human ARPKD gene.","volume":"13","author":"Nagasawa","year":"2002","journal-title":"J Am Soc Nephrol"},{"key":"R11-20-20241201","doi-asserted-by":"crossref","first-page":"2703","DOI":"10.1093\/hmg\/ddg274","article-title":"Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia.","volume":"12","author":"Ward","year":"2003","journal-title":"Hum Mol Genet"},{"key":"R13-20-20241201","doi-asserted-by":"crossref","first-page":"2570","DOI":"10.1681\/ASN.2009020188","article-title":"Cystin localizes to primary cilia via membrane microdomains and a targeting motif.","volume":"20","author":"Tao","year":"2009","journal-title":"J Am Soc Nephrol"},{"key":"R15-20-20241201","doi-asserted-by":"crossref","first-page":"1657","DOI":"10.1038\/sj.emboj.7600160","article-title":"A transcriptional network in polycystic kidney disease.","volume":"23","author":"Gresh","year":"2004","journal-title":"EMBO J"},{"key":"R16-20-20241201","doi-asserted-by":"crossref","first-page":"814","DOI":"10.1172\/JCI200420083","article-title":"Mutation of hepatocyte nuclear factor-1beta inhibits Pkhd1 gene expression and produces renal cysts in mice.","volume":"113","author":"Hiesberger","year":"2004","journal-title":"J Clin Invest"},{"key":"R17-20-20241201","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1093\/hmg\/9.5.769","article-title":"Germline and somatic loss of function of the mouse cpk gene causes biliary ductal pathology that is genetically modulated.","volume":"9","author":"Guay-Woodford","year":"2000","journal-title":"Hum Mol Genet"},{"key":"R18-20-20241201","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1172\/JCI0214099","article-title":"Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease.","volume":"109","author":"Hou","year":"2002","journal-title":"J Clin Invest"},{"key":"R19-20-20241201","doi-asserted-by":"crossref","first-page":"556","DOI":"10.1016\/S0022-5347(17)53911-7","article-title":"Murine congenital polycystic kidney disease: a model for studying development of cystic disease.","volume":"127","author":"Preminger","year":"1982","journal-title":"J Urol"},{"key":"R20-20-20241201","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1186\/1476-5926-8-5","article-title":"Immunohistochemical study of the phenotypic change of the mesenchymal cells during portal tract maturation in normal and fibrous (ductal plate malformation) fetal liver.","volume":"8","author":"Villeneuve","year":"2009","journal-title":"Comp Hepatol"},{"key":"R21-20-20241201","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1038\/ki.2009.440","article-title":"Genotype-phenotype correlations in fetuses and neonates with autosomal recessive polycystic kidney disease.","volume":"77","author":"Denamur","year":"2010","journal-title":"Kidney Int"},{"key":"R22-20-20241201","doi-asserted-by":"crossref","first-page":"2077","DOI":"10.1242\/jcs.01067","article-title":"Shh-dependent differentiation of intestinal tissue from embryonic pancreas by activin A.","volume":"117","author":"van Eyll","year":"2004","journal-title":"J Cell Sci"},{"key":"R23-20-20241201","doi-asserted-by":"crossref","first-page":"1849","DOI":"10.1101\/gad.340305","article-title":"Control of liver cell fate decision by a gradient of TGF beta signaling modulated by Onecut transcription factors.","volume":"19","author":"Clotman","year":"2005","journal-title":"Genes Dev"},{"key":"R24-20-20241201","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1016\/j.jpeds.2006.12.006","article-title":"Neonatal cholestatic jaundice as the first symptom of a mutation in the hepatocyte nuclear factor-1beta gene (HNF-1beta).","volume":"150","author":"Beckers","year":"2007","journal-title":"J Pediatr"},{"key":"R25-20-20241201","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1007\/BF02075756","article-title":"Paucity of intrahepatic bile ducts, solitary kidney and atrophic pancreas with diabetes mellitus: atypical Alagille syndrome?","volume":"155","author":"Devriendt","year":"1996","journal-title":"Eur J Pediatr"},{"key":"R26-20-20241201","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1681\/ASN.2008020238","article-title":"HNF-1beta regulates transcription of the PKD modifier gene Kif12.","volume":"20","author":"Gong","year":"2009","journal-title":"J Am Soc Nepthrol"},{"key":"R27-20-20241201","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1136\/jmg.2007.054999","article-title":"Recent advances in the molecular pathology, cell biology and genetics of ciliopathies.","volume":"45","author":"Adams","year":"2008","journal-title":"J Med Genet"},{"key":"R28-20-20241201","doi-asserted-by":"crossref","first-page":"1472","DOI":"10.1091\/mbc.e06-09-0848","article-title":"Liver progenitor cells develop cholangiocyte-type epithelial polarity in three-dimensional culture.","volume":"18","author":"Tanimizu","year":"2007","journal-title":"Mol Biol Cell"},{"key":"R29-20-20241201","doi-asserted-by":"crossref","first-page":"2486","DOI":"10.1091\/mbc.e08-02-0177","article-title":"Liver progenitor cells fold up a cell monolayer into a double-layered structure during tubular morphogenesis.","volume":"20","author":"Tanimizu","year":"2009","journal-title":"Mol Biol Cell"}],"container-title":["Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fhep.24292","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/hep.24292","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/hep.24292","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.lww.com\/01515467-201106000-00020","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,1]],"date-time":"2024-12-01T13:24:13Z","timestamp":1733059453000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/01515467-201106000-00020"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,6]]},"references-count":27,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2011]]}},"URL":"https:\/\/doi.org\/10.1002\/hep.24292","relation":{},"ISSN":["0270-9139"],"issn-type":[{"value":"0270-9139","type":"print"}],"subject":[],"published":{"date-parts":[[2011,6]]}}}