Proliferation-independent role of NF2 (merlin) in limiting biliary morphogenesis.

ABSTRACT: The architecture of individual cells and cell collectives enables functional specification, a prominent example being the formation of epithelial tubes that transport fluid or gas in many organs. The intrahepatic bile ducts (IHBDs) form a tubular network within the liver parenchyma that transports bile to the intestine. Aberrant biliary 'neoductulogenesis' is also a feature of several liver pathologies including tumorigenesis. However, the mechanism of biliary tube morphogenesis in development or disease is not known. Elimination of the neurofibromatosis type 2 protein (NF2; also known as merlin or neurofibromin 2) causes hepatomegaly due to massive biliary neoductulogenesis in the mouse liver. We show that this phenotype reflects unlimited biliary morphogenesis rather than proliferative e

SUBMITTER: Benhamouche-Trouillet S 

PROVIDER: S-EPMC10682933 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature