International Journal of Hepatology / 2012 / Article / Tab 1

Review Article

Pathological Features of New Animal Models for Primary Biliary Cirrhosis

Table 1

Requirements for the ideal animal model of PBC.

(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

(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)

(iii) Other immunological characters
 (1) Increase in inflammatory cytokines
 (2) Decrease in functional regulatory T cells
 (3) Increase in natural killer T (NKT) cells

(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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