Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic inflammatory autoimmune cholestatic liver disease whose diagnosis is based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies (AMA) or specific ANA reactivity (anti-sp100 and anti-gp210) and histologic evidence of chronic non-suppurative, granulomatous small bile duct cholangitis [103, 104]. Here, NUP210 is linked to primary biliary cholangitis.