2017 Fiscal Year Final Research Report
Novel therapeutic approaches for biliary cystogenesis of Caroli's disease with congenital hepatic fibrosis based on the analysis of microRNAs
Project/Area Number |
15K08342
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Human pathology
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Research Institution | Kanazawa University |
Principal Investigator |
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Project Period (FY) |
2015-04-01 – 2018-03-31
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Keywords | 肝臓 / 胆道 / 分子病理 |
Outline of Final Research Achievements |
The polycystic kidney (PCK) rat was analyzed as an animal model of Caroli’s disease with congenital hepatic fibrosis in this study. In vitro experiments revelated that PCK cholangiocytes were characterized by the reduced expression of miR-125b-1-3p, and the resultant overexpression of Smoothened and the Hedgehog signaling activation led to overgrowth of PCK cholangiocytes. In vivo administration of Smoothened antagonist (cyclopamine) significantly reduced the severity of bile duct dilatation and kidney cyst formation of the PCK rats. These results indicate that inhibition of the Hedgehog signaling represents a potential target of therapy for Caroli’s disease with congenital hepatic fibrosis.
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Free Research Field |
人体病理学
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