There are 2 binding motifs (A and B boxes) in HMGB1, and the active cytokine domain of HMGB1 is localized to box B.31 HMGB1 plays a role in virus-induced biliary atresia pathogenesis and acts as a target for therapeutic intervention in some patients with biliary atresia and high HMGB1 expression.32 We found that higher HMGB1 expression persisted from biliary proliferation, atypical cystic hyperplasia, and cholangiomas to CCA. This evidence concerns the gene HMGB1 and biliary atresia.