Supporting data for “Dysregulation of Methionine Metabolism of Hepatocytes Induced Epithelial-mesenchymal Transition of Hepatocytes and Abnormal Growth of Cholangiocytes in Biliary Atresia”

This research related to the hepatocytes and cholangiocyte organoids derived from iPSCs. These iPSC cell lines were derived from peripheral blood of patient with MAT1A mutation(BA638C) and a healthy control(HKUPS001). The specific MAT1A mutation was corrected to generate an iPSC cell line share the same genetic background with BA638C. Cholangiocyte organoids were also generated from the liver tissue of biliary atresia and non-biliary atresia patients.

This specific MAT1A mutation worked as a entry point to study the methionine metabolic dysfunction of hepatocytes and its relation to the biliary atresia pathogenesis. The data included in this item are the relevant sequencing results of hepatocytes and cholangiocyte organoids derived from the iPSC cell lines descrived above.

The results of this study indicates that (i) the MAT1A mutation induced EMT-like behavior of hepatocytes, potentially promoting liver fibrosis in disease progression; (ii) patients’ hepatocytes induced abnormal cholangiocyte development, which suggests that hepatocyte metabolic dysfunction can lead to cholangiocyte/bile duct injury in the initiation of BA.