By Observing the Effects of Alagille Syndrome, Researchers Discover How Liver Tissue Can Transform and Heal

According to a story from PR Newswire, a group of scientists have managed to discover a mechanism that allows the liver to repair damage it has sustained. This discovery may be able to reduce the need for organ transplants, which are costly and can often be difficult to find. This new discovery was found thanks to the observation of a mouse model of Alagille syndrome.
Alagille syndrome is a genetic disorder that causes changes to a variety of systems and organs in the body, such as the kidneys, liver, and and heart. The effects of Alagille syndrome usually appear in infancy or early childhood. The disorder is very rare but is also probably under diagnosed, as the symptoms can be so mild that they can go unnoticed. Symptoms can include lack of bile secretion, jaundice, enlarged spleen, itchy skin, cholesterol deposits on the skin, heart murmurs, heart defects, pulmonary stenosis, and more. People with the syndrome often share distinctive facial features, such as prominent forehead, a small, pointed chin, and deeply set eyes. There are several treatments available that can improve bile flow, but surgery may be necessary to treat heart problems, and a liver transplant is often recommended later in life. To learn more about Alagille syndrome, click here.

In the animal model, the mice were born without a complete biliary tree, a network of vessels that allows bile to flow into the intestine as needed. In Alagille syndrome, patients may have almost all of the biliary tree missing or may have one that has narrow vessels or is otherwise underdeveloped. It has long been understood that the liver had an exceptional ability to repair itself compared to other organs, but it was unclear how this happened. The liver needs this ability because of its essential role in processing the substances that people ingest.

In the study, once the mice reached adulthood, their biliary tree was nearly the same as an unaffected mouse. This suggests that cells in the liver, that were originally of one kind of tissue, were able to transform in order to form the biliary tree passages that were originally missing. Further research confirmed that the cells transforming was the primary mechanism for repair.

More research will be needed in order to determine which transcription factor proteins allow for this transformation to take place, but the researchers definitely see the potentially in using this mechanism to treat disease and reduce the need for organ transplants.

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