In the healthy liver, cholangiocytes contribute to bile secretion via release of bicarbonate and water.
Cholangiocytes act through bile-acid independent bile flow, which is driven by the active transport of electrolytes.
This results in passive transcellular and paracellular secretion of fluid and electrolytes through an osmotic effect.
Disordered functioning of cholangiocytes is at the center of a variety of pathological conditions known as "cholangiopathies."
These diseases include primary biliary cirrhosis, primary sclerosing cholangitis, AIDS cholangiopathy, disappearing bile duct syndromes, Alagille's syndrome, cystic fibrosis, and biliary atresia.