Table 1.
Human PBC | NOD.c3c4 | 2445 | dnTGFβRII | IL-2Rα−/− | |
---|---|---|---|---|---|
General feature | |||||
Genetic factor | 60% concordance in identical twin | 100% | 100% | 100% | 100% |
Environment factor | Yes | ? | ? | ? | ? |
Affected gender | mostly female | both sexes | both sexes | both sexes | both sexes |
Age of onset | 40–60 ys | 9–10 wks | 9–10 wks | 5–6 wks | 5–6 wks |
Liver histology | |||||
specific destruction of small bile ducts | Yes | Yes | Yes | Yes | Yes |
periductual T cell infiltration | Yes | Yes | Yes | Yes | Yes |
Granuloma formation | Yes | Yes | Yes | No | No |
Eosinophilia | Yes | Yes | Yes | No | No |
Fibrosis | Yes | No | No | No | No |
Pathological role of lymphocytes | ? | Yes* | ? | Yes* | ? |
T cell immunity | |||||
liver infiltration of T cells | Yes | Yes | Yes | Yes | Yes |
autoreactive CD4+ T cell response | Yes | ? | Yes | Yes | ? |
target of autoreactive CD4+ T cells | PDC-E2 | ? | PDC-E2 | PDC-E2 | ? |
reduced Treg frequency | Yes | ? | ? | ? | Yes |
increased iNKT frequency | Yes | ? | ? | Yes | No |
B cell immunity | |||||
Immunoglobulins | IgM, IgG | IgM, IgA | ? | IgM, IgG, IgA | IgA, IgG |
AMA | Yes | Yes | Yes | Yes | Yes |
Dominant AMA target protein | PDC-E2 | PDC-E2 | PDC-E2 | PDC-E2 | PDC-E2 |
Dominant Epitope | Lipoyl domain | Lipoyl domain | Lipoyl domain | Lipoyl domain | Lipoyl domain |
ANA | Yes | Yes | No | ? | Yes |
Serum biochemistry | |||||
increased alkaline phosphatase | Yes | ? | ? | ? | ? |
serum cytokines | |||||
increased IFN-γ | Yes | Yes | ? | Yes | Yes |
increased TNF-α | Yes | Yes | ? | Yes | Yes |
adoptive transfer of splenocytes causes disease.