Biliary organoids uncover delayed epithelial development and barrier function in biliary atresia.

ABSTRACT:

Background and aims

Biliary atresia is a severe inflammatory and fibrosing cholangiopathy of neonates of unknown etiology. The onset of cholestasis at birth implies a prenatal onset of liver dysfunction. Our aim was to investigate the mechanisms linked to abnormal cholangiocyte development.

Approach and results

We generated biliary organoids from liver biopsies of infants with biliary atresia and normal and diseased controls. Organoids emerged from biliary atresia livers and controls and grew as lumen-containing spheres with an epithelial lining of cytokeratin-19^pos albumin^neg SOX17^neg cholangiocyte-like cells. Spheres had similar gross morphology in all three groups and expressed cholangiocyte-enriched genes. In biliary atresia, cholangiocyt

SUBMITTER: Amarachintha SP 

PROVIDER: S-EPMC9983428 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature