. Author manuscript; available in PMC: 2009 May 1.

Published in final edited form as: Clin Liver Dis. 2008 May;12(2):333–ix. doi: 10.1016/j.cld.2008.02.011

Table 1.

Human PBC vs. ABD mouse models: Clinical, histological and immunological observations.

	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.