Primary biliary cirrhosis (PBC) is a slowly progressive, chronic liver disease characterized histologically by nonsuppurative destruction of intrahepatic bile duct epithelial cells and serologically by the presence of disease-specific antimitochondrial antibodies (AMAs) and antinuclear antibodies (ANA), targeting the sp100 and gp210 antigens [1–3]. The gene discussed is NUP210; the disease is primary biliary cholangitis.