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Home  > Press Release  > Loss of Fibrocystin Promotes Interleukin-8-Dependent Proliferation and CTGF Production of Biliary Epithelium

Loss of Fibrocystin Promotes Interleukin-8-Dependent Proliferation and CTGF Production of Biliary Epithelium

Abstract

Congenital hepatic fibrosis (CHF) is a genetic liver disease with abnormal proliferation of cholangiocyte and progressive hepatic fibrosis. Mutation of polycystic kidney and hepatic disease 1 (PKHD1) causes CHF and the dysfunction of its coding protein, fibrocystin, is associated with ductal plate malformation during fetal development and further pathological changes in CHF patients. However, the underlying molecular mechanism of CHF remains unclear, which is quite different from liver cirrhosis. This study investigated the molecular mechanism of CHF-pathophysiology using a genetically engineered human induced pluripotent stem (iPS) cell model to aid the discovery of novel therapeutic agents for CHF.

Journal Article

JOURNAL:
Journal of Hepatology

TITLE:
Loss of Fibrocystin Promotes Interleukin-8-Dependent Proliferation and CTGF Production of Biliary Epithelium

DOI:
https://doi.org/10.1016/j.jhep.2019.02.024

Correspondence to

Sei KAKINUMA,M.D., Ph.D.,Associate Professor
Department of Liver Disease Control,
Graduate School of Medical and Dental Sciences,
Tokyo Medical and Dental University(TMDU)
E-mail:skakinuma.gast(at)tmd.ac.jp