Article
A Novel Pkhd1 Mutation Interacts with the Nonobese Diabetic Genetic Background To Cause Autoimmune Cholangitis.
Journal of immunology (Baltimore, Md. : 1950) - 1 Jan 2018
Huang Wenting, Rainbow Daniel B, Wu Yuehong, Adams David, Shivakumar Pranavkumar, Kottyan Leah, Karns Rebekah, Aronow Bruce, Bezerra Jorge, Gershwin M Eric, Peterson Laurence B, Wicker Linda S, Ridgway William M
Abstract excerpt
We previously reported that NOD.c3c4 mice develop spontaneous autoimmune biliary disease (ABD) with anti-mitochondrial Abs, histopathological lesions, and autoimmune T lymphocytes similar to human primary biliary cholangitis. In this article, we demonstrate that ABD in NOD.c3c4 and related NOD ABD strains is caused by a chromosome 1 region that includes a novel mutation in polycystic kidney and hepatic disease 1...
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