title: Analysis of biliary epithelial-mesenchymal transition in portal tract fibrogenesis in biliary atresia.
abstract: BACKGROUND
  The cellular origin of myofibroblast in the liver fibrosis remains unclear. This study was designed to investigate whether biliary epithelial cells (BECs) undergoing epithelial-mesenchymal transition (EMT) might be found in patients with biliary atresia, thereby serving as a source of fibrotic myofibroblasts.
  
  
  METHODS
  Liver sections from patients with biliary atresia were evaluated to detect antigen for the BECs marker 4 and cytokeratin-7 (CK-7), proteins (fibroblast-specific protein 1, also known S100A4; the collagen chaperone heat shock protein 47, HSP47) characteristically expressed by cells undergoing EMT, as well as myofibroblasts marker a-smooth muscle actin (a-SMA).
  
  
  RESULTS
  Normal bile ducts BECs could express CK-7 and low levels of a-SMA; they did not express S100A4 and HSP47. However, BECs from biliary atresia resulted in increased expression of a-SMA, S100A4, with concurrent transition to a fibroblast-like morphology and decreased expression of AK-7. Furthermore, BECs in biliary atresia were associated with significant bile ductular proliferation and coexpressed both epithelial and mesenchymal markers.
  
  
  CONCLUSIONS
  From significant histologic evidence, the BECs forming small- and medium-sized bile ducts undergoing EMT may account for prominent bile ductular proliferation and directly contribute to fibrogenesis in BA.
authors: Department of Hepatobiliary Surgery, Children's Hospital of Chongqing Medical University, 136 Zhongshan 2nd Rd., Chongqing, 400014, People's Republic of China.
authors: Yu-Hua
authors: YH
authors: Deng
authors: Cong-Lun
authors: CL
authors: Pu
authors: Ying-Cun
authors: YC
authors: Li
authors: Jin
authors: J
authors: Zhu
authors: Chunping
authors: C
authors: Xiang
authors: Ming-Man
authors: MM
authors: Zhang
authors: Chun-Bao
authors: CB
authors: Guo
mesh_terms: D000199:Actins / Q000378:metabolism; D001652:Bile Ducts / Q000473:pathology*; D001656:Biliary Atresia / Q000378:metabolism / Q000473:pathology*; D049109:Cell Proliferation; D058750:Epithelial-Mesenchymal Transition; D050958:HSP47 Heat-Shock Proteins / Q000378:metabolism; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053552:Keratin-7 / Q000378:metabolism; D008103:Liver Cirrhosis / Q000378:metabolism / Q000473:pathology*; D058628:Myofibroblasts / Q000378:metabolism / Q000473:pathology*; D011168:Portal System / Q000378:metabolism / Q000473:pathology; D000071999:S100 Calcium-Binding Protein A4; D009418:S100 Proteins / Q000378:metabolism
chemical_list: D000199:Actins; D050958:HSP47 Heat-Shock Proteins; D053552:Keratin-7; D000071999:S100 Calcium-Binding Protein A4; D009418:S100 Proteins; C494660:SERPINH1 protein, human; C077591:S100A4 protein, human