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Notch Signaling is Essential to Modulate Intrahepatic Bile Duct Structure

Cholangiopathies, or diseases which affect the biliary epithelial cells of the liver, are an important health concern in the United States. Specifically, the diagnosis of a cholangiopathy is the number one indicator of pediatric liver transplant and the number three indicator for adults. A common clinical finding in cholangiopathies is cholestasis or reduced bile flow, however the specific pathogenesis of most cholangiopathies is largely unknown.
A subtype of cholangiopathy, which this research focuses on, is the genetic cholangiopathy. One specific example of a genetic cholangiopathy is Alagille syndrome, a pleiotropic disorder primary characterized by a lack of bile ducts. Alagille syndrome is highly associated with mutations in the Notch signaling pathway. In this dissertation, I use mouse models that modulate Notch signaling to define the developmental and post-natal mechanism of cholestasis in a cholangiopathy model.

Identiferoai:union.ndltd.org:VANDERBILT/oai:VANDERBILTETD:etd-03102011-141048
Date14 March 2011
CreatorsSparks, Erin Elizabeth
ContributorsJames Goldenring, Kathleen Gould, Mark Magnuson, Guoqiang Gu, Stacey Huppert
PublisherVANDERBILT
Source SetsVanderbilt University Theses
LanguageEnglish
Detected LanguageEnglish
Typetext
Formatapplication/pdf
Sourcehttp://etd.library.vanderbilt.edu/available/etd-03102011-141048/
Rightsrestricted, I hereby certify that, if appropriate, I have obtained and attached hereto a written permission statement from the owner(s) of each third party copyrighted matter to be included in my thesis, dissertation, or project report, allowing distribution as specified below. I certify that the version I submitted is the same as that approved by my advisory committee. I hereby grant to Vanderbilt University or its agents the non-exclusive license to archive and make accessible, under the conditions specified below, my thesis, dissertation, or project report in whole or in part in all forms of media, now or hereafter known. I retain all other ownership rights to the copyright of the thesis, dissertation or project report. I also retain the right to use in future works (such as articles or books) all or part of this thesis, dissertation, or project report.

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