Animal Models of Primary Biliary Cirrhosis
Tomohiro Katsumi, Kyoko Tomita, Patrick S Leung, Guo xiang Yang, M. Eric Gershwin, Yoshiyuki Ueno
Rheumatology, Allergy, and Clinical Immunology
Primary biliary cirrhosis (PBC) is characterized histologically by the presence of chronic non-suppurative destructive cholangitis of the small interlobular bile duct, leading to chronic progressive cholestasis. Most PBC patients are asymptomatic and have a reasonable prognosis, but a few develop esophageal varices or jaundice, rapidly leading to liver failure within a short period. As multiple factors appear to be involved in the onset of PBC, its clinical course may be complicated. Therefore, the use of an animal model would be valuable for clarifying the pathogenesis of PBC. Here, we review recent data of selected PBC models, particularly spontaneous models, xenobiotic immunized models, and infection-triggered models. There are a number of spontaneous models: the NOD.c3c4, dominant-negative TGF-β receptor II, IL-2Rα−/−, Scurfy, and Ae2a,b−/− mice. These animal models manifest distinct clinical and immunological features similar, but also often different, from those of human PBC. It is clear that a combination of genetic predisposition, environmental factors, and immunological dysfunction contribute to the pathogenesis of PBC. The diverse clinical course and complexity of the immunological mechanisms of PBC cannot be fully recapitulated solely any single animal model. The challenge remains to develop a progressive PBC disease model that exhibits fibrosis, and ultimately hepatic failure.
Clinical Reviews in Allergy and Immunology
Genetically modified spontaneous models
Infection-triggered model
Many derivation models
Xenobiotic immunized animal models
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Biliary Liver Cirrhosis Medicine & Life Sciences 100%
Animal Models Medicine & Life Sciences 60%
Liver Failure Medicine & Life Sciences 20%
Cholangitis Medicine & Life Sciences 11%
Esophageal and Gastric Varices Medicine & Life Sciences 11%
Cholestasis Medicine & Life Sciences 10%
Xenobiotics Medicine & Life Sciences 10%
Immunologic Factors Medicine & Life Sciences 10%
Katsumi, T., Tomita, K., Leung, P. S., Yang, G. X., Gershwin, M. E., & Ueno, Y. (2015). Animal Models of Primary Biliary Cirrhosis. Clinical Reviews in Allergy and Immunology, 48(2-3), 142-153. https://doi.org/10.1007/s12016-015-8482-y
Animal Models of Primary Biliary Cirrhosis. / Katsumi, Tomohiro; Tomita, Kyoko; Leung, Patrick S; Yang, Guo xiang; Gershwin, M. Eric; Ueno, Yoshiyuki.
In: Clinical Reviews in Allergy and Immunology, Vol. 48, No. 2-3, 01.06.2015, p. 142-153.
Katsumi, T, Tomita, K, Leung, PS, Yang, GX, Gershwin, ME & Ueno, Y 2015, 'Animal Models of Primary Biliary Cirrhosis', Clinical Reviews in Allergy and Immunology, vol. 48, no. 2-3, pp. 142-153. https://doi.org/10.1007/s12016-015-8482-y
Katsumi T, Tomita K, Leung PS, Yang GX, Gershwin ME, Ueno Y. Animal Models of Primary Biliary Cirrhosis. Clinical Reviews in Allergy and Immunology. 2015 Jun 1;48(2-3):142-153. https://doi.org/10.1007/s12016-015-8482-y
Katsumi, Tomohiro ; Tomita, Kyoko ; Leung, Patrick S ; Yang, Guo xiang ; Gershwin, M. Eric ; Ueno, Yoshiyuki. / Animal Models of Primary Biliary Cirrhosis. In: Clinical Reviews in Allergy and Immunology. 2015 ; Vol. 48, No. 2-3. pp. 142-153.
@article{ab7ccc52be8b495abc12abc51f866e37,
title = "Animal Models of Primary Biliary Cirrhosis",
abstract = "Primary biliary cirrhosis (PBC) is characterized histologically by the presence of chronic non-suppurative destructive cholangitis of the small interlobular bile duct, leading to chronic progressive cholestasis. Most PBC patients are asymptomatic and have a reasonable prognosis, but a few develop esophageal varices or jaundice, rapidly leading to liver failure within a short period. As multiple factors appear to be involved in the onset of PBC, its clinical course may be complicated. Therefore, the use of an animal model would be valuable for clarifying the pathogenesis of PBC. Here, we review recent data of selected PBC models, particularly spontaneous models, xenobiotic immunized models, and infection-triggered models. There are a number of spontaneous models: the NOD.c3c4, dominant-negative TGF-β receptor II, IL-2Rα−/−, Scurfy, and Ae2a,b−/− mice. These animal models manifest distinct clinical and immunological features similar, but also often different, from those of human PBC. It is clear that a combination of genetic predisposition, environmental factors, and immunological dysfunction contribute to the pathogenesis of PBC. The diverse clinical course and complexity of the immunological mechanisms of PBC cannot be fully recapitulated solely any single animal model. The challenge remains to develop a progressive PBC disease model that exhibits fibrosis, and ultimately hepatic failure.",
keywords = "Autoimmunity, Environmental factors, Genetically modified spontaneous models, Infection-triggered model, Many derivation models, Xenobiotic immunized animal models",
author = "Tomohiro Katsumi and Kyoko Tomita and Leung, {Patrick S} and Yang, {Guo xiang} and Gershwin, {M. Eric} and Yoshiyuki Ueno",
journal = "Clinical Reviews in Allergy and Immunology",
publisher = "Humana Press",
T1 - Animal Models of Primary Biliary Cirrhosis
AU - Katsumi, Tomohiro
AU - Tomita, Kyoko
AU - Leung, Patrick S
AU - Yang, Guo xiang
AU - Gershwin, M. Eric
AU - Ueno, Yoshiyuki
N2 - Primary biliary cirrhosis (PBC) is characterized histologically by the presence of chronic non-suppurative destructive cholangitis of the small interlobular bile duct, leading to chronic progressive cholestasis. Most PBC patients are asymptomatic and have a reasonable prognosis, but a few develop esophageal varices or jaundice, rapidly leading to liver failure within a short period. As multiple factors appear to be involved in the onset of PBC, its clinical course may be complicated. Therefore, the use of an animal model would be valuable for clarifying the pathogenesis of PBC. Here, we review recent data of selected PBC models, particularly spontaneous models, xenobiotic immunized models, and infection-triggered models. There are a number of spontaneous models: the NOD.c3c4, dominant-negative TGF-β receptor II, IL-2Rα−/−, Scurfy, and Ae2a,b−/− mice. These animal models manifest distinct clinical and immunological features similar, but also often different, from those of human PBC. It is clear that a combination of genetic predisposition, environmental factors, and immunological dysfunction contribute to the pathogenesis of PBC. The diverse clinical course and complexity of the immunological mechanisms of PBC cannot be fully recapitulated solely any single animal model. The challenge remains to develop a progressive PBC disease model that exhibits fibrosis, and ultimately hepatic failure.
AB - Primary biliary cirrhosis (PBC) is characterized histologically by the presence of chronic non-suppurative destructive cholangitis of the small interlobular bile duct, leading to chronic progressive cholestasis. Most PBC patients are asymptomatic and have a reasonable prognosis, but a few develop esophageal varices or jaundice, rapidly leading to liver failure within a short period. As multiple factors appear to be involved in the onset of PBC, its clinical course may be complicated. Therefore, the use of an animal model would be valuable for clarifying the pathogenesis of PBC. Here, we review recent data of selected PBC models, particularly spontaneous models, xenobiotic immunized models, and infection-triggered models. There are a number of spontaneous models: the NOD.c3c4, dominant-negative TGF-β receptor II, IL-2Rα−/−, Scurfy, and Ae2a,b−/− mice. These animal models manifest distinct clinical and immunological features similar, but also often different, from those of human PBC. It is clear that a combination of genetic predisposition, environmental factors, and immunological dysfunction contribute to the pathogenesis of PBC. The diverse clinical course and complexity of the immunological mechanisms of PBC cannot be fully recapitulated solely any single animal model. The challenge remains to develop a progressive PBC disease model that exhibits fibrosis, and ultimately hepatic failure.
KW - Autoimmunity
KW - Environmental factors
KW - Genetically modified spontaneous models
KW - Infection-triggered model
KW - Many derivation models
KW - Xenobiotic immunized animal models
JO - Clinical Reviews in Allergy and Immunology
JF - Clinical Reviews in Allergy and Immunology