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. Here, PDHX is linked to primary biliary cholangitis.