Like >90% of PBC patients, these mice develop autoreactive T-cell and B-cell responses against the dihydrolipoyl acetyltransferase (E2) and dihydrolipoyl dehydrogenase-binding protein (E3BP) components of the PDC complex10–12, leading to biliary epithelial cell destruction, cholestasis, small bile duct proliferation, and liver failure. This evidence concerns the gene PDHX and liver failure.