Data from: Liver and biliary disease in mice with ulcerative colitis-like colon inflammation

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  • Primary sclerosing cholangitis (PSC) is a rare inflammatory disease of the liver and biliary tract occurs in a small subset of in patients inflammatory bowel disease (IBD), particularly those with ulcerative colitis (UC). There are no approved therapies for PSC and this disease often progresses to liver failure. However, development of effective treatments is currently hampered by both the lack of mechanistic understanding of early stages of PSC pathogenesis and by the lack of murine models that closely recapitulate the clinical findings in humans. Triple knockout 'TIA' mice that are deficient in tumor necrosis factor--α (TNF), interleukin (IL)-10, and activation-induced cytidine deaminase (Aicda) spontaneously develop severe microbiota-dependent UC-like colitis soon after weaning. We report here that these TIA mice also develop inflammation of the liver and biliary tract that is suggestive of autoimmune hepatitis biochemically, with liver and biliary lesions including bile ductular proliferation, interface hepatitis, and inflammatory obliteration or fibrosis of bile ducts. Oral treatment with the novel therapeutic remogliflozin etabonate (Remo) for 7 weeks markedly decreased the development and progression of liver and biliary disease in TIA mice, by decreasing inflammatory cell infiltration, bile ductular proliferation, and interface hepatitis. Given their clinical and histologic similarities, we hypothesize that study of the liver and biliary disease that arises in TIA mice with UC-like colitis may provide insights relevant to early pathogenesis and progression of UC-associated liver diseases in humans. Further studies will be needed to establish how closely late stage disease in TIA mice models human PSC and to determine the mechanisms responsible for the beneficial therapeutic effects of Remo in this model. ... [Read More]

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4 files (168 KB)
Data Citation
  • Hale, L. (2021). Data from: Liver and biliary disease in mice with ulcerative colitis-like colon inflammation. Duke Research Data Repository. https://doi.org/10.7924/r4ws8r786
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  • 10.7924/r4ws8r786
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  • ark:/87924/r4ws8r786
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Collection Dates
  • 2016-2018
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Related Materials
  • manuscript under consideration at PLOS One
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  • NIH
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  • CA-170069
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  • Data from: Liver and biliary disease in mice with ulcerative colitis-like colon inflammation
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file details ReadMe.txt 2021-06-09 Public Download
file details data for Tables 2 & 4.xlsx 2021-06-09 Public Download
file details data for Table 3.xlsx 2021-06-09 Public Download
file details data for Fig2 & S1 Fig.xlsx 2021-06-09 Public Download

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