The developmental origins of Notch-driven intrahepatic bile duct disorders

Author:

Martinez Lyons Anabel1,Boulter Luke1ORCID

Affiliation:

1. MRC Human Genetics Unit, Institute of Genetics and Cancer, Edinburgh EH4 2XU, UK

Abstract

ABSTRACT The Notch signaling pathway is an evolutionarily conserved mechanism of cell–cell communication that mediates cellular proliferation, cell fate specification, and maintenance of stem and progenitor cell populations. In the vertebrate liver, an absence of Notch signaling results in failure to form bile ducts, a complex tubular network that radiates throughout the liver, which, in healthy individuals, transports bile from the liver into the bowel. Loss of a functional biliary network through congenital malformations during development results in cholestasis and necessitates liver transplantation. Here, we examine to what extent Notch signaling is necessary throughout embryonic life to initiate the proliferation and specification of biliary cells and concentrate on the animal and human models that have been used to define how perturbations in this signaling pathway result in developmental liver disorders.

Funder

Cancer Research UK

Publisher

The Company of Biologists

Subject

General Biochemistry, Genetics and Molecular Biology,Immunology and Microbiology (miscellaneous),Medicine (miscellaneous),Neuroscience (miscellaneous)

Cited by 2 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. The importance of figures in scientific ‘show and tell’;Disease Models & Mechanisms;2023-11-01

2. NOTCH signalling – a core regulator of bile duct disease?;Disease Models & Mechanisms;2023-08-22

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