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
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
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