Immune-mediated Cholangiohepatitis in Neonatally Thymectomized Mice: the Role of T Cells and Analysis of T-Cell Receptor Vβ Usage

Aisaka, Yasuyuki; Watanabe, Yoshifumi; Kamiyasu, Masaya; Masanaga, Toshiyuki; Tsuji, Keiji; Nakanishi, Toshio; Kajiyama, Goro; Gershwin, M. Eric

doi:10.1006/jaut.2000.0368

Cited by 6 publications

(1 citation statement)

References 23 publications

(22 reference statements)

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“…Masanaga et al in 1998 used PDC to immunize A/J mice that were neonatally thymectomized, and they succeeded in inducing pathognomonic cholangitis/biliary damage. Although the bile duct lesion in this model was similar to that of human PBC, the appearance of AMA was not clearly described, and the removal of the thymus in the neonatal period posed as a technical difficulty [15, 16]. Tsuneyama et al [17] and Ohba et al [18] reported the presence of AMA in the serum of MRL/ lpr mice, a model of autoimmune disease in which vasculitis, glomerular nephritis, arthritis, inflammation of the salivary glands, and interstitial pneumonia develop spontaneously in the same individual.…”

Section: Previously Reported Pbc Animal Models Of Pbc (Up To 2005)mentioning

confidence: 99%

Pathological Features of New Animal Models for Primary Biliary Cirrhosis

Tsuneyama

Moritoki

Kikuchi

et al. 2012

International Journal of Hepatology

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Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by immune mediated biliary damage and frequent appearance of autoantibodies against mitochondrial enzymes. There is almost no useful animal model that is globally recognized and routinely used, however, several unique animal models manifested the characteristic clinical and pathological features of human PBC within the last 5 years. Herein, we compare the pathological features of previously reported and newly introduced novel animal models of PBC. Knowledge and understanding of the strengths and the limitations of each animal model have led to the development of promising therapies and novel tools to characterize these clinical conditions. Moreover, suitability of the model for the intended purpose should be confirmed by further research and analysis.

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Section: Previously Reported Pbc Animal Models Of Pbc (Up To 2005)mentioning

confidence: 99%

Pathological Features of New Animal Models for Primary Biliary Cirrhosis

Tsuneyama

Moritoki

Kikuchi

et al. 2012

International Journal of Hepatology

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Animal Models of Biliary Disease: Current Approaches and Limitations

Pollheimer

Fickert

2017

Biliary Disease

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Animal Models in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

Pollheimer

Fickert

2014

Clinic Rev Allerg Immunol

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Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are immune-mediated cholangiopathies with enigmatic etiology and pathogenesis. They have distinct clinical, laboratory, immunological, and histomorphological characteristics. Well-characterized animal models for PBC and PSC are utterly needed to develop novel pathogenetic concepts and to study innovative treatment strategies. The aim of the current paper is to outline the characteristics of ideal PBC and PSC animal models and to contrast this with a real-life up-to-date overview of currently available mouse models. Although some of this models show several individual characteristics of PBC and PSC, it is obvious that all of them have substantial and important limitations. Nevertheless, some may be beneficial to study certain pathophysiological aspects. Potential cholangiopathy animal models should be systematically investigated in regard to elevated serum alkaline phosphatase, bilirubin, and bile acid levels; immunological abnormalities; and longitudinal studies in regard to their liver phenotype. We herein propose a common systematic workup for potential models based on the fact that there are some intriguing disease combinations in specific genetically modified mice and recommend a stepwise process in regard to model characterization with methodical harvesting and screening of numerous organs for potential concomitant diseases. Due to the complex nature of both cholangiopathies, it seems to be very likely that no single perfect PBC or PSC model will ever be generated. The models outlined herein will certainly help to clarify specific pathogenetic aspects and even more important may turn out to be suitable to test potential drugs for treatment.

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Immune-mediated Cholangiohepatitis in Neonatally Thymectomized Mice: the Role of T Cells and Analysis of T-Cell Receptor Vβ Usage

Cited by 6 publications

References 23 publications

Pathological Features of New Animal Models for Primary Biliary Cirrhosis

Pathological Features of New Animal Models for Primary Biliary Cirrhosis

Animal Models of Biliary Disease: Current Approaches and Limitations

Animal Models in Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

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