Abstract
higher prevalence of bile duct obstruction in these mice (number of mice with obstructed/patent bile ducts: 9/2 in ABCB4 vs 3/11 in wt, p = 0.005). Furthermore, frequencies of hepatic CD8 and of NK lymphocytes were both higher in ABCB4 compared with wt mice (%CD8+CD3+/lymphocytes: 10.3 vs 6.2, p = 0.03; %NK+CD3−/lymphocytes: 12.7 vs 7.3%, p = 0.002), as determined by flow cytometry. Interestingly, enhanced lymphocyte activation was accompanied by 25-fold up-regulation of hepatic iNOS mRNA expression in these mice. Conclusion: An infant with EHBA had anatomical and molecular features of ABCB4/MDR3 deficiency. In a murine model, ABCB4 heterozygosity enhances hepatic CD8 and NK lymphocyte responses to virus infection and increases the frequency of ductal obstruction. Whether this activation is causally linked to hepatic up-regulation of iNOS and low phospholipid “toxic bile” requires further investigation.
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