Pathological Features of New Animal Models for Primary Biliary Cirrhosis

Table 1

Requirements for the ideal animal model of PBC.

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(i) Specific liver pathology (cellular immunity)

 (1) Destruction of interlobular bile duct

 (2) T-cell aggregation around the damaged bile ducts

 (3) Epithelioid granuloma formation

 (4) Fibrosis/cirrhosis

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(ii) Specific autoantibodies (humoral immunity)

 (1) Antimitochondrial autoantibodies (AMAs)

 (2) Anti-PDC-E2 antibodies, anti-BCOADC-E2 antibodies,  and anti-OGDC-E2 antibodies

 (3) Antinuclear antibodies (ANAs)

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(iii) Other immunological characters

 (1) Increase in inflammatory cytokines

 (2) Decrease in functional regulatory T cells

 (3) Increase in natural killer T (NKT) cells

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(iv) General versatility

 (1) High reproducibility and disease frequency

 (2) Simplicity of model production

 (3) Long-term maintenance of disease

 (4) Long lifespan without severe complicating disorders

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