hum0486-v1Release info

Bile duct development and its failure mechanisms using biliary atresia and Alagille syndrome-specific iPS cells

Aims

Biliary atresia (BA) is a poor prognosis disease that causes fibrotic obstruction of the extrahepatic bile ducts by early infancy and progresses from bile stagnation to cirrhosis, the cause of which is still unknown. The aim of this study is to establish a bile duct cell induction system from normal human iPS cells and to prove the abnormality during the fetal period. Using this system, we will elucidate the molecular mechanisms of bile duct formation after mid-development, establish stable long-term culture techniques for bile duct stem cells, construct bile duct tree structures through in vitro and in vivo experiments, and establish an experimental system to investigate the bile duct injury process using biliary atresia-specific iPS cells.

Methods

whole genome sequencing (WGS)

Participants/materials

iPS cell samples generated from five patients with biliary atresia (type I cyst)

URL

• https://acd.brc.riken.jp/en/ (opens in a new tab)

Principal investigator

Yohei Hayashi

Affiliation

RIKEN BioResource Research Center

No grants.

No use of the controlled access data has been recorded.