title: Role of cholangiocytes in primary biliary cirrhosis.
abstract: Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by selective destruction of intrahepatic cholangiocytes. Mechanisms underlying the development and progression of the disease are still controversial and largely undefined. Evidence suggests that PBC results from an articulated immunologic response against an immunodominant mitochondrial autoantigen, the E2 component of the pyruvate dehydrogenase complex (PDC-E2); characteristics of the disease are also the presence of disease-specific antimitochondrial autoantibodies (AMAs) and autoreactive CD4 and CD8 T cells. Recent evidence suggests that cholangiocytes show specific immunobiological features that are responsible for the selective targeting of those cells by the immune system. The immune reaction in PBC selectively targets small sized, intrahepatic bile ducts; although a specific reason for that has not been defined yet, it has been established that the biliary epithelium displays a unique heterogeneity, for which the physiological and pathophysiological features of small and large cholangiocytes significantly differ. In this review article, the authors provide a critical overview of the current evidence on the role of cholangiocytes in the immune-mediated destruction of the biliary tree that characterizes PBC.
authors: Liver Unit and Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Rozzano (MI), Italy.
authors: Ana
authors: A
authors: Lleo
authors: Clinic of Gastroenterology and Hepatology, Università Politecnica delle Marche, Ancona, Italy.
authors: Luca
authors: L
authors: Maroni
authors: Research, Central Texas Veterans Health Care System, S and W and Texas A and M System Health Science Center, College of Medicine, Temple, Texas.
authors: Shannon
authors: S
authors: Glaser
authors: Research, Central Texas Veterans Health Care System, S and W and Texas A and M System Health Science Center, College of Medicine, Temple, Texas.
authors: Gianfranco
authors: G
authors: Alpini
authors: Clinic of Gastroenterology and Hepatology, Università Politecnica delle Marche, Ancona, Italy.
authors: Marco
authors: M
authors: Marzioni
mesh_terms: D000818:Animals; D017209:Apoptosis; D015551:Autoimmunity; D001653:Bile Ducts, Intrahepatic / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D049109:Cell Proliferation; D004195:Disease Models, Animal; D004847:Epithelial Cells / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D015398:Signal Transduction