Congenital biliary atresia is an incurable disease of newborn infants that is characterised by deformation of the gallbladder and biliary duct system. Yoshiakira Kanai and co-workers now report (p. 639) that haploinsufficiency of Sox17 in C57BL/6 background mice provides a genetic model for this poorly understood condition. The researchers show that SOX17, a transcription factor that is required for definitive endoderm development in various vertebrate species, is expressed at the distal edge of the gallbladder primordium during gallbladder and bile duct development. In Sox17+/− C57BL/6 embryos, cell-autonomous defects in the proliferation and maintenance of the gallbladder/bile duct epithelia lead to epithelial cell detachment from the luminal wall, bile duct atresia (blockage), bile leakage and inflammation in the bile ducts and liver at late foetal stages. These results suggest that SOX17 has a dose-dependent function in the morphogenesis and maturation of gallbladder and bile duct epithelia during late organogenesis and provide new insights into the pathogenesis of congenital biliary atresia.
Unpaired Sox17 models biliary atresia
Unpaired Sox17 models biliary atresia. Development 1 February 2013; 140 (3): e302. doi:
Download citation file:
Advertisement
Cited by
Call for papers: Uncovering Developmental Diversity
Development invites you to submit your latest research to our upcoming special issue: Uncovering Developmental Diversity. This issue will be coordinated by our academic Editor Cassandra Extavour (Harvard University, USA) alongside two Guest Editors: Liam Dolan (Gregor Mendel Institute of Molecular Plant Biology, Austria) and Karen Sears (University of California Los Angeles, USA).
Choose Development in 2024
In this Editorial, Development Editor-in-Chief James Briscoe and Executive Editor Katherine Brown explain how you support your community by publishing in Development and how the journal champions serious science, community connections and progressive publishing.
Journal Meeting: From Stem Cells to Human Development
Register now for the 2024 Development Journal Meeting From Stem Cells to Human Development. Early-bird registration deadline: 3 May. Abstract submission deadline: 21 June.
Pluripotency of a founding field: rebranding developmental biology
This collaborative Perspective, the result of a workshop held in 2023, proposes a set of community actions to increase the visibility of the developmental biology field. The authors make recommendations for new funding streams, frameworks for collaborations and mechanisms by which members of the community can promote themselves and their research.
Read & Publish Open Access publishing: what authors say
We have had great feedback from authors who have benefitted from our Read & Publish agreement with their institution and have been able to publish Open Access with us without paying an APC. Read what they had to say.