Antigen	M.W. (kD)*	LD	Frequency (%) in PBC†	Inhibition of B-Cell Epitopes (Amino acid residues)		Major Ig Isotype	Function by AMA
PDC-E2	74	+	90-95	Outer and inner LD	(128-221)	IgG3, IgM	+
E3BP	55	+	90-95	LD	NR	IgG	NR
BCOADC-E2	52	+	53-55	LD	(1-84)	NR	+
OGDC-E2	48	+	39-88	LD	(67-147)	IgG2, IgM	+
PDC-E1	41	-	41-66	TTP binding and phosphorylation		NR	+
				Sites	NR

*Determined by sodium dodecyl sulfate-polyacrylamide gel (SDS-PAGE) and immunoblotting against PBC sera.

†Determined by immunoblotting or enzyme-linked immunosorbent assay (ELISA) against recombinant proteins

LD, lipoyl domain; NR, not reported.

Antigen	Amino Acid Sequence	CD4+ T Cell Response*
	CD8+ T-cell epitope
PDC-E2 (ILD)	157-176	G E K L S E G D L L A E I E T D K A T I	+
PDC-E2 (OLD)	30-49	G D K I N E G D L I A E V E T D K A T V	+
OGDC-E2	94-113	G D T V A E D E V V C E I E T D K T S V	+
BCOADC-E2	89-108	G D T V S Q F D S I C E V Q S D K A S V	NR
E3 binding protein	71-90	G E A V S A G D A L C E I E T D K A V V	NR
PDC-E2 (E. coli)	31-44/134-147/235-24	E Q S L I T V E G D K A S M +
BCOADC-E2 (Pseudomonas putida)	104-117	D E L L A T I E T D K I D I 2
HLA DR chain	82-95	Q G A L A N I A V D K A N L 2
Human glycogen phosphorylase	354-367	L V D L E R M D W D K A W D 2

Underlines indicate identical or homologous amino acids.

ILD, inner lipoyl domain; OLD, outer lipoyl domain; NR, not reported.

*human PDC-E2 163-176-specific CD4+ T cells were used as responsive T cells.

	Normal	PBC	Control Liver Diseases
Autoantigen
PDC-E2[49,50]	+	+++*	+
PDC-E3BP[51]	+	+++*	+
Anion-exchanger-2[60]	+	-~+	+
Immune adhesion molecules
MHC class I[35]	+	++†	+~++
MHC class II (HLA-DR)[35,36]	-	++†	+
CD1d	-	+	-
ICAM-1 (CD54)[36-38]	-	-~+	-
VCAM-1[38]	-	-~+	-
LFA-3 (CD58)[39]	-	-~+	-~+
B7-1 (CD80)[39,43]	-	-~+?	-
B7-2 (CD86)[42,43]	-	-~+?	-~+
Apoptosis-related molecules
Fas (CD95)[63]	-	++†	-~+
Granzyme B[63]	-	++	-~+
Perforin[63]	+	++†	++
Bcl-2[62]	+	++†	+
Inflammatory cytokines[44]
IL-6	-~+	+~++	+
TNF-	-~+	+~++	+
IL-6 receptor	-	-~+	-
TNF receptor	-~+	+~++	+
Others
MUC1 apomucin	-	+†	+†
MUC6 apomucin	-~	+†	+†
Lewis Y antigen[63]	-~+	++†	+†
Heat shock protein[60,70]	-~+	++	-~+

*Increased apical staining.

†Increased expression in damaged bile ducts.