Cholangiocytes are the

epithelial cell Epithelium or epithelial tissue is a thin, continuous, protective layer of Cell (biology), cells with little extracellular matrix. An example is the epidermis, the outermost layer of the skin. Epithelial (Mesothelium, mesothelial) tissues line ...

s of the

bile duct A bile duct is any of a number of long tube-like structures that carry bile, and is present in most vertebrates. The bile duct is separated into three main parts: the fundus (superior), the body (middle), and the neck (inferior). Bile is requ ...

. They are cuboidal epithelium in the small interlobular bile ducts, but become columnar and

carbonate A carbonate is a salt of carbonic acid, (), characterized by the presence of the carbonate ion, a polyatomic ion with the formula . The word "carbonate" may also refer to a carbonate ester, an organic compound containing the carbonate group ...

-secreting in larger bile ducts approaching the porta hepatis and the extrahepatic ducts. They contribute to

hepatocyte A hepatocyte is a cell of the main parenchymal tissue of the liver. Hepatocytes make up 80% of the liver's mass. These cells are involved in: * Protein synthesis * Protein storage * Transformation of carbohydrates * Synthesis of cholesterol, bi ...

survival by transporting

bile acid Bile acids are steroid acids found predominantly in the bile of mammals and other vertebrates. Diverse bile acids are synthesized in the liver in peroxisomes. Bile acids are conjugated with taurine or glycine residues to give anions called bile ...

Function

In the healthy liver, cholangiocytes contribute to

bile Bile (from Latin ''bilis''), also known as gall, is a yellow-green/misty green fluid produced by the liver of most vertebrates that aids the digestion of lipids in the small intestine. In humans, bile is primarily composed of water, is pro ...

secretion via release of

bicarbonate In inorganic chemistry, bicarbonate (IUPAC-recommended nomenclature: hydrogencarbonate) is an intermediate form in the deprotonation of carbonic acid. It is a polyatomic anion with the chemical formula . Bicarbonate serves a crucial bioche ...

and water. Several hormones and locally acting mediators are known to contribute to cholangiocyte fluid/electrolyte secretion. These include

secretin Secretin is a hormone that regulates water homeostasis throughout the body and influences the environment of the duodenum by regulating secretions in the stomach, pancreas, and liver. It is a peptide hormone produced in the S cells of the duodenum ...

acetylcholine Acetylcholine (ACh) is an organic compound that functions in the brain and body of many types of animals (including humans) as a neurotransmitter. Its name is derived from its chemical structure: it is an ester of acetic acid and choline. Par ...

, ATP, and bombesin. Cholangiocytes act through bile-acid independent bile flow, which is driven by the active transport of electrolytes. In contrast, hepatocytes secrete bile through bile-acid dependent bile flow, which is coupled to canalicular secretion of bile acids via ATP-driven transporters. This results in passive transcellular and paracellular secretion of fluid and electrolytes through an osmotic effect.

Clinical significance

Disordered functioning of cholangiocytes is at the center of a variety of pathological conditions known as "cholangiopathies." These diseases include

primary biliary cirrhosis Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to buil ...

primary sclerosing cholangitis Primary sclerosing cholangitis (PSC) is a long-term progressive disease of the liver and gallbladder characterized by inflammation and scarring of the bile ducts, which normally allow bile to drain from the gallbladder. Affected individuals may ...

, AIDS cholangiopathy, disappearing bile duct syndromes, Alagille's syndrome,

cystic fibrosis Cystic fibrosis (CF) is a genetic disorder inherited in an autosomal recessive manner that impairs the normal clearance of Sputum, mucus from the lungs, which facilitates the colonization and infection of the lungs by bacteria, notably ''Staphy ...

, and

biliary atresia Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired. ...

. Often either the best or the only curative treatment option for cholangiopathies is

liver transplantation Liver transplantation or hepatic transplantation is the replacement of a diseased liver with the healthy liver from another person (allograft). Liver transplantation is a treatment option for end-stage liver disease and acute liver failure, al ...

, and as a group, cholangiopathies account for a substantial percentage of both adult and pediatric liver transplants.

Research

Active scientific investigation of cholangiocytes focuses on such diverse processes as mechanisms of fluid/electrolyte secretion, regulation of cholangiocyte proliferation, roles of cholangiocytes in the pathogenesis of liver fibrosis and cirrhosis, and cholangiocyte apoptosis. Specific investigation of individual cholangiopathies is also pursued actively.

References

{{Authority control Epithelial cells Digestive system Human cells