| Notch signaling regulates bile duct morphogenesis in mice. | |
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MedLine Citation:
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PMID: 18365007 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Alagille syndrome is a developmental disorder caused predominantly by mutations in the Jagged1 (JAG1) gene, which encodes a ligand for Notch family receptors. A characteristic feature of Alagille syndrome is intrahepatic bile duct paucity. We described previously that mice doubly heterozygous for Jag1 and Notch2 mutations are an excellent model for Alagille syndrome. However, our previous study did not establish whether bile duct paucity in Jag1/Notch2 double heterozygous mice resulted from impaired differentiation of bile duct precursor cells, or from defects in bile duct morphogenesis. METHODOLOGY/PRINCIPAL FINDINGS: Here we characterize embryonic biliary tract formation in our previously described Jag1/Notch2 double heterozygous Alagille syndrome model, and describe another mouse model of bile duct paucity resulting from liver-specific deletion of the Notch2 gene. CONCLUSIONS/SIGNIFICANCE: Our data support a model in which bile duct paucity in Notch pathway loss of function mutant mice results from defects in bile duct morphogenesis rather than cell fate specification. |
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Authors:
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Julie Lozier; Brent McCright; Thomas Gridley |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural Date: 2008-03-26 |
Journal Detail:
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Title: PloS one Volume: 3 ISSN: 1932-6203 ISO Abbreviation: PLoS ONE Publication Date: 2008 |
Date Detail:
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Created Date: 2008-03-26 Completed Date: 2008-08-08 Revised Date: 2009-11-18 |
Medline Journal Info:
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Nlm Unique ID: 101285081 Medline TA: PLoS One Country: United States |
Other Details:
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Languages: eng Pagination: e1851 Citation Subset: IM |
Affiliation:
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The Jackson Laboratory, Bar Harbor, Maine, United States of America. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Bile Ducts / growth & development* Heterozygote Immunohistochemistry Mice Morphogenesis* Receptors, Notch / genetics, metabolism* Signal Transduction* |
| Grant Support | |
ID/Acronym/Agency:
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DK066387/DK/NIDDK NIH HHS; NS036437/NS/NINDS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Receptors, Notch |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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