The characteristics of PBC are sustained elevation (>6 months) above the ULN for serum ALP activity, the presence of frequently granulomatous inflammation of the portal tracts accompanying lymphocytic mediated damage to (and destruction of) the small intrahepatic bile ducts, with accompanying cholestasis, and a typical pattern of serum and secretory autoantibodies reactive predominantly with mitochondrial antigens (AMA; reactivity with PBC-specific antinuclear antibodies (ANA) is also seen). Here, BTG3 is linked to primary biliary cholangitis.