PBC: Animal Models of Cholangiopathies and Possible Endogenous Viral Infections

Primary Biliary Cirrhosis (PBC) is considered an autoimmune disease characterized by immune-mediated destruction of the intrahepatic bile ducts and its characteristic serologic marker, the anti-mitochondrial antibody (AMA). Several factors were proposed to clarify the pathological and immunological...

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Main Authors: Masashi Ninomiya, Yoshiyuki Ueno, Tooru Shimosegawa
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:International Journal of Hepatology
Online Access:http://dx.doi.org/10.1155/2012/649290
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author Masashi Ninomiya
Yoshiyuki Ueno
Tooru Shimosegawa
author_facet Masashi Ninomiya
Yoshiyuki Ueno
Tooru Shimosegawa
author_sort Masashi Ninomiya
collection DOAJ
description Primary Biliary Cirrhosis (PBC) is considered an autoimmune disease characterized by immune-mediated destruction of the intrahepatic bile ducts and its characteristic serologic marker, the anti-mitochondrial antibody (AMA). Several factors were proposed to clarify the pathological and immunological mechanisms of PBC. Immunological reaction with a bacterial or a viral association was identified in the previous report, and it seems probable that PBC was thought to have such an etiology. The majority of patients with PBC was reported to have both RT-PCR and immunohistochemistry evidence of human betaretrovirus infection in lymph nodes or in 2008, the patient who developed PBC with high HIV viral load had an antiviral therapy and recovered. To understand the etiology of PBC associated with infection, several factors should be considered and especially animal models may be useful. In this paper, we introduce three typical animal models of PBC: the dominant-negative form of transforming growth factor-β receptor type II (dnTGFβRII) mouse, IL-2Rα−/− mouse and NOD.c3c4 mouse, are enumerated and described, and we discuss previous reports of viral infection associated with PBC and consider the etiology of PBC from our analysis of results in NOD.c3c4 mouse.
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spelling doaj-art-f26bdbcf88e54394b6017121df04201c2025-02-03T05:46:05ZengWileyInternational Journal of Hepatology2090-34482090-34562012-01-01201210.1155/2012/649290649290PBC: Animal Models of Cholangiopathies and Possible Endogenous Viral InfectionsMasashi Ninomiya0Yoshiyuki Ueno1Tooru Shimosegawa2Division of Gastroenterology, Department of Gastroenterology, Tohoku University Graduate School of Medicine, Seiryo, Aoba-ku, Sendai 980-8575, JapanDivision of Gastroenterology, Department of Gastroenterology, Tohoku University Graduate School of Medicine, Seiryo, Aoba-ku, Sendai 980-8575, JapanDivision of Gastroenterology, Department of Gastroenterology, Tohoku University Graduate School of Medicine, Seiryo, Aoba-ku, Sendai 980-8575, JapanPrimary Biliary Cirrhosis (PBC) is considered an autoimmune disease characterized by immune-mediated destruction of the intrahepatic bile ducts and its characteristic serologic marker, the anti-mitochondrial antibody (AMA). Several factors were proposed to clarify the pathological and immunological mechanisms of PBC. Immunological reaction with a bacterial or a viral association was identified in the previous report, and it seems probable that PBC was thought to have such an etiology. The majority of patients with PBC was reported to have both RT-PCR and immunohistochemistry evidence of human betaretrovirus infection in lymph nodes or in 2008, the patient who developed PBC with high HIV viral load had an antiviral therapy and recovered. To understand the etiology of PBC associated with infection, several factors should be considered and especially animal models may be useful. In this paper, we introduce three typical animal models of PBC: the dominant-negative form of transforming growth factor-β receptor type II (dnTGFβRII) mouse, IL-2Rα−/− mouse and NOD.c3c4 mouse, are enumerated and described, and we discuss previous reports of viral infection associated with PBC and consider the etiology of PBC from our analysis of results in NOD.c3c4 mouse.http://dx.doi.org/10.1155/2012/649290
spellingShingle Masashi Ninomiya
Yoshiyuki Ueno
Tooru Shimosegawa
PBC: Animal Models of Cholangiopathies and Possible Endogenous Viral Infections
International Journal of Hepatology
title PBC: Animal Models of Cholangiopathies and Possible Endogenous Viral Infections
title_full PBC: Animal Models of Cholangiopathies and Possible Endogenous Viral Infections
title_fullStr PBC: Animal Models of Cholangiopathies and Possible Endogenous Viral Infections
title_full_unstemmed PBC: Animal Models of Cholangiopathies and Possible Endogenous Viral Infections
title_short PBC: Animal Models of Cholangiopathies and Possible Endogenous Viral Infections
title_sort pbc animal models of cholangiopathies and possible endogenous viral infections
url http://dx.doi.org/10.1155/2012/649290
work_keys_str_mv AT masashininomiya pbcanimalmodelsofcholangiopathiesandpossibleendogenousviralinfections
AT yoshiyukiueno pbcanimalmodelsofcholangiopathiesandpossibleendogenousviralinfections
AT toorushimosegawa pbcanimalmodelsofcholangiopathiesandpossibleendogenousviralinfections