The hallmarks of PBC diagnosis are the serum autoimmune antibodies, including anti-mitochondrial antibodies (AMAs, >95% positive in PBC patients) that target the pyruvate dehydrogenase complex E2 subunit (PDC-E2), and anti-nuclear antibodies (ANAs), the 210-kDa glycoprotein of the nuclear pore complex (anti-gp210), and the nuclear antigen Sp100 (anti-sp100) (Courvalin et al., 1990; Sternsdorf et al., 1995; Hu et al., 2014; Rodrigues et al., 2018). Here, NUP210 is linked to primary biliary cholangitis.