Next to a spontaneous production of antimitochondrial antibodies (AMAs) directed to the same mitochondrial autoantigens as in human disease (for example, the E2 component of the pyruvate dehydrogenase complex (PDC-E2), the E2 subunit of the branched chain 2-oxo-acid dehydrogenase complex (BCOADC-E2) and the E2 subunit of the 2-oxo-glutarate dehydrogenase complex (OGDC-E2)), these mice show a lymphocytic liver infiltration with periportal inflammation similar to histological changes in human PBC. This evidence concerns the gene DLAT and primary biliary cholangitis.