Carolis Disease Current Knowledge of Its Biliary Pathogenesis Obtained from an Orthologous Rat Model英文文献资料.docVIP

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Carolis Disease Current Knowledge of Its Biliary Pathogenesis Obtained from an Orthologous Rat Model英文文献资料.doc

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Carolis Disease Current Knowledge of Its Biliary Pathogenesis Obtained from an Orthologous Rat Model英文文献资料

HindawiPublishingCorporation InternationalJournalofHepatology Volume2012,ArticleID107945,10pages doi:10.1155/2012/107945 ReviewArticle Caroli’sDisease:CurrentKnowledgeofItsBiliaryPathogenesis ObtainedfromanOrthologousRatModel YasunoriSato,XiangShanRen,andYasuniNakanuma DepartmentofHumanPathology,GraduateSchoolofMedicine,KanazawaUniversity,Kanazawa920-8640,Japan CorrespondenceshouldbeaddressedtoYasunoriSato,sato-ya@med.kanazawa-u.ac.jp Received23March2011;Accepted7April2011 AcademicEditor:YoshiyukiUeno Copyright?2012YasunoriSatoetal.ThisisanopenaccessarticledistributedundertheCreativeCommonsAttributionLicense, whichpermitsunrestricteduse,distribution,andreproductioninanymedium,providedtheoriginalworkisproperlycited. Caroli’s disease belongs to a group of hepatic ?bropolycystic diseases and is a hepatic manifestation of autosomal recessive polycystic kidney disease (ARPKD). It is a congenital disorder characterized by segmental saccular dilatations of the large intrahepatic bile duct and is frequently associated with congenital hepatic ?brosis (CHF). The most viable theory explaining its pathogenesis suggests that it is related to ductal plate malformation. The development of the polycystic kidney (PCK) rat, an orthologous rodent model of Caroli’s disease with CHF as well as ARPKD, has allowed the molecular pathogenesis of the disease and the therapeutic options for its treatment to be examined. The relevance of the ?ndings of studies using PCK rats and/or the cholangiocyte cell line derived from them to the pathogenesis of human Caroli’s disease is currently being analyzed. Fibrocystin/polyductin, the gene product responsible for ARPKD, is normally localized to primary cilia, and defects in the ?brocystin from primary cilia are observed in PCK cholangiocytes. Ciliopathies involving PCK cholangiocytes (cholangiociliopathies)appeartobeassociatedwithdecreasedintracellularcalciumlevelsandincreasedcAMPconcentrations, causingcholangiocytehyperproliferation,abnormalcellma