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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

Research Project

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Project/Area Number 15K08342
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Human pathology
Research InstitutionKanazawa University

Principal Investigator

Sato Yasunori  金沢大学, 医学系, 准教授 (30324073)

Project Period (FY) 2015-04-01 – 2018-03-31
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.

Free Research Field

人体病理学

URL: 

Published: 2019-03-29  

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