Table 1 Comparison of the characteristics of animal models of primary biliary cholangitis (PBC)
From: Animal models of primary biliary cholangitis: status and challenges
Characteristics | dnTGF-βRII mice | NOD.c3c4 mice | AE2a,b−/− mice | ARE-Del−/− mice | IL-2Rα−/− mice | Scurfy mice | 2-OA immunised mice | E. coli infected mice | BDP immunised mice |
|---|---|---|---|---|---|---|---|---|---|
Mouse strain | C57BL/6 | NOD | FVB/N | C57BL/6 | C57BL/6 | C57BL/6 | C57BL/6 | NOD.B6-Idd10/Idd18 | C57BL/6 |
Modelling principle | Abrogation of TGF-β signalling in T cells | Acquisition of insulin-dependent diabetes resistance | Dysregulation of intracellular pH in BEC and disruption of the "biliary bicarbonate umbrella" | Long-term and chronic IFN-γ overexpression | Treg dysfunction | Defective Treg function | Mimic of the lipoic acid-lysine located in the PDC-E2 domain | Cross-recognition induced by the PDC-E2 epitope | Breakdown of immune tolerance |
Trigger | Transgenic | Transgenic | Transgenic | Transgenic | Transgenic | Transgenic | 2-OA | E. coli | BDP |
Experimental period | 4–40 weeks | 9–30 weeks | 3–15 months | 8–40 weeks | 4–28 weeks | 3–4 weeks | 4–24 weeks after immunisation | 4–26 weeks after infection | 1 week after immunisation |
Serological features | |||||||||
 ALP | —— | —— |  +  ~  +  +  | —— | —— | —— | —— | —— | —— |
 ANA | 100% | 80 ~ 90% | —— | —— | 80% | —— | —— | —— | —— |
 AMA | 100% | 50 ~ 60% | 80% | 100% | 100% | 100% | 100% | 100% | 100% |
 Immunoglobulin | IgM, IgA, IgG | IgM,IgG | IgM,IgG | IgM,IgG | IgA, IgG | IgM, IgA, IgG | IgM, IgA, IgG | —— | —— |
Histological features | |||||||||
 Granuloma | —— |  +  | —— |  +  | —— | —— |  +  | —— | —— |
 Eosinophilia | —— |  +  |  +  | —— | —— |  +  | —— | —— | —— |
 Lymphocytic inflammation |  +  +  +  |  +  +  +  |  +  ~  +  +  +  |  +  +  +  |  +  +  +  |  +  +  ~  +  +  +  |  +  ~  +  +  |  +  |  +  |
 Bile duct destruction |  +  +  |  +  |  +  ~  +  +  +  |  +  ~  +  +  |  +  ~  +  +  |  +  ~  +  +  |  +  ~  +  +  |  +  | —— |
 Liver fibrosis | —— |  ~  +  |  ~  +  |  +  | —— | —— | —— | —— | —— |