Etiopathogenesis of primary biliary cirrhosis

Table 1 Immunopathological characteristics of biliary epithelium in PBC[90]

	Normal	PBC
Expression level of PDC-E2	+	+ + +
Adhesion molecules
- ICAM-1	+	+ +
- VCAM-1	-/+	+
- LFA-1	-/+	+
- E-selectin		+ +
Biliary intra-epithelial lymphocytes	Large bile ducts, few CD4+	Small bile ducts,increased CD4+CD28-
Apoptosis-related molecules
- Fas (CD95)	-	+
- granzyme B	-	-/+
- perforin	-	-/+
- bcl-2	+ +	-
BEC phagocytosis of apoptotic BECs	-	+ +
Cytokines
- INF-γ	-	+ +
- IL-2	-	+ +
- IL-6	-	+ +
- IL-6 receptor	-	-/+
- TNF-α	-/+	+ +
- TNF receptor	-/+	+ +

Table 2 Mitochondrial and nuclear autoantigens in PBC

	Autoantigens
Mitochondrial antigens	E2 subunits of 2-OADC	PDC-E2
		OGDC-E2
		BCOADC-E2
	Pyruvate dehydrogenase	E3BP
	complex	PDC E1α
Nuclear antigens	Nuclear pore complex	gp210
		nucleoporin 62
	Multiple nuclear dots	Sp100
		PML
	Anticentromere

2-OADC: 2-oxo-acid dehydrogenase complex; PDC: Pyruvate dehydrogenase complex; OGDC: Oxoglutarate dehydrogenase complex; BCOADC: Branched chain 2-oxo-acid dehydrogenase complex; E3BP: Dihydrolipoamide dehydrogenase (E3)-binding protein.