TABLE 1.

Summary of autoantibodies, the respective target antigens, and clinical utility in autoimmune cholestatic liver conditions

Antibody	Target antigen	Liver condition	Clinical utility
Anti-mitochondrial Ab (AMA)	Components of oxidative phosphorylation chain (i.e., PDC-E2, KGDC)	PBC	Diagnosis of PBC in combination with cholestatic liver enzymes
Anti-gp210	Transmembrane nuclear pore complex	PBC	Diagnosis of AMA negative PBC. Some studies correlate presence with poor prognosis
Anti-Tpr	Nuclear basket inner membrane of nucleus	PBC	Associated with PBC and other autoimmune conditions
Anti-centromere Ab	Centromere-kinetochore protein complex	PBC	Associated with PBC and other autoimmune conditions
Anti PML, sp100, sp140	Domains within the nuclear matrix	PBC	Diagnosis of AMA negative PBC. Some studies correlate presence with poor prognosis
Anti Hexokinase	Hexokinase located in outer membrane of mitochondria	PBC	Associated with both AMA positive and AMA negative PBC not yet validated for prognostication
Anti-Kelch-like 12	Collagen export and ubiquitination nuclear protein	PBC	Associated with both AMA positive and AMA negative PBC not yet validated for prognostication
Antineutrophil Cytoplasmic Ab	Cytoplasmic and perinuclear proteins of neutrophils and monocyte lysosomes	PSC	Limited diagnostic and prognostic use
Immunoglobulin G4	Various antigens are described with different organ involvement	IgG4-related dissases	Elevated serum levels with imaging and histology are diagnostic for IgG4 related disease