Type-I but not type-II interferon receptor knockout mice are susceptible to biliary atresia

Pediatric Research
Joachim F KueblerC Petersen

Abstract

The etiology of biliary atresia (BA) is not yet understood, but recent studies have shown inflammation with an up-regulated interferon (IFN) activity in the intra- and extrahepatic bile ducts of patients with BA. These findings support an inflammatory/infectious cause of BA as mimicked in our infective murine model. To study the role of the IFN receptors in our model, we used mice with inactivated INF-alpha/beta receptor A129, with inactivated IFN-gamma receptor G129, or inactivation of both interferon receptors AG129 as well as the wild type controls W129. Mice were infected with rotavirus within 48h of birth and 7 d postpartum. The incidence of BA in each group was determined during a 3 wk period. In the second week the virus load was measured. BA incidence was 76% in A129 and 67% in AG129 animals, whereas in the G129 group only 33% of the pups developed BA. The wild type presented with a BA-incidence of 15%, while 7 d old mice failed to develop BA. There was no significant difference in the virus load of the livers between the groups independent of clinical symptoms. In conclusion, inactivation of type I INF-receptor significantly increases the incidence of BA following postpartal rotavirus infection. This effect is independ...Continue Reading

References

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Citations

Aug 31, 2013·Orphanet Journal of Rare Diseases·Claus Petersen, Mark Davenport
Jul 18, 2012·Seminars in Pediatric Surgery·Claus Petersen
Nov 17, 2009·Lancet·Jane L HartleyDeirdre A Kelly
Sep 5, 2014·Clinical Pediatrics·Dong ZhaoQiang Xia
Nov 30, 2011·Current Opinion in Gastroenterology·Paula M Hertel, Mary K Estes
Apr 4, 2018·Innovative Surgical Sciences·Claus Petersen, Omid Madadi-Sanjani
Sep 19, 2018·Bulletin of Experimental Biology and Medicine·V I KonenkovT I Dergacheva

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