Researchers highlight the mechanism of biliary repair, including cholangiocyte proliferation, progenitor cell differentiation, and hepatocyte-cholangiocyte transdifferentiation, and its role in biliary diseases such as immune-mediated cholangiopathies, inherited cholangiopathies like Alagille syndrome (ALGS), obstructive cholangiopathies, and cholangiocarcinoma. They published their results in Frontiers in Immunology.

The ultimate aims of the review were to “deepen the understanding of the mechanisms of biliary repair” and provide “potential novel therapeutic methods for treating biliary diseases.,” the authors said.

Focusing on inherited cholangiopathies such as ALGS, the authors say that understanding how biliary repair is regulated during these diseases is very important since knowledge about their pathogenesis is elusive and effective treatments are scarce. Such an understanding can allow a better understanding of the diseases themselves and the development of potential new therapies for them.

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For example, it is known that liver progenitor cell differentiation plays a major role in ALGS and is mainly regulated by Notch signaling. Hepatocyte-cholangiocyte transdifferentiation is also involved in the development of the disease.

“Many kinds of animal mutation models have been developed to exploit the role of the biliary system,” the authors wrote. “However, few studies have focused on the crosstalk of immune cells and surrounding cells in the development of ALGS.”

The study also highlights the role of immune cells such as Kupffer cells, monocytes, lymphocytes, neutrophils, and mast cells in biliary repair in cholangiopathies, as well as potential new techniques in biliary repair such as lineage tracing techniques, single-cell related techniques, and liver organoids.

“Although accumulating studies have proven dynamic biliary repair during cholangiopathies, the contributions of different regenerating pools of cholangiocytes remain an open question,” they concluded. “More novel techniques remain needed to clarify the underlying mechanisms of biliary repair.”


Lan T, Qian S, Tang C, Gao J. Role of immune cells in biliary repair. Front Immunol. 2022;13:866040. doi:10.3389/fimmu.2022.866040