Most PBC-specific AMAs bind to a trypsin-sensitive antigen of the inner mitochondrial membrane previously named M2 (6), mainly targeting the E2 component that forms the core structure of the pyruvate dehydrogenase complex (PDC) in the majority of patients with PBC, but the E1 and E3 subunits are also recognized (7), along with E3BP (initially known as “protein X”) (8). Here, PDHX is linked to primary biliary cholangitis.