Pathologically, PBC is characterized by highly conserved humoral and cellular autoreactive immune responses to the mitochondrial pyruvate dehydrogenase complex E2 (PDC-E2).23, 24 This loss of tolerance has been attributed to the aberrant expression of molecular mimics of PDC-E2 on the cell surface of biliary epithelial cells (BEC), which behave as immunodominant epitopes and bind with HLA-DRB4.25 This evidence concerns the gene DLAT and primary biliary cholangitis.