Table 2.
Characteristics of PBC mouse models.
| Spontaneous models |
Induced model |
||||
|---|---|---|---|---|---|
| NOD.c3c4 | dnTGFbRII | IL-2Rα −/− | ARE Del−/− | 2-OA–BSA immunized | |
| Female dominance | Yes | No | No | Yes | No |
| Cholestasis | – | + | – | + | + |
| AMA seropositivity | 50–60% | 100% | 100% | 100% | 100% |
| Portal inflammation | +++ | +++ | +++ | Yes | + |
| Granulomas | + | − | − | + | + |
| Other features | Biliary polycystic lesions | Moderate colitis | Severe anemia, inflammatory bowel diseases, and short life span | Peritonitis | |
| References | (58) | (59) | (60) | (61,62) | (55) |
PBC: primary biliary cholangitis; 2-OA: 2-octynoic acid; BSA: bovine serum albumin; ARE: adenylate uridine-rich element; AMA: anti-mitochondrial autoantibody.