Mouse Models of Primary Biliary Cirrhosis

Author(s): Axel R. Concepcion, Juan F. Medina

Journal Name: Current Pharmaceutical Design

Volume 21 , Issue 18 , 2015

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Primary biliary cirrhosis (PBC) is a chronic and progressive cholestatic liver disease of unknown etiopathogenesis that mainly affects middle-aged women. Patients show non-suppurative cholangitis with damage and destruction of small- and medium-sized intrahepatic bile ducts. Characteristically, the disease is strongly associated with autoimmune phenomena such as the appearance of serum antimitochondrial autoantibodies (AMA) and portal infiltrates with autoreactive T cells which recognize the inner lipoyl domain of the E2 component of the pyruvate dehydrogenase complex (PDC-E2). Here we review the major characteristics of a series of inducible and genetically modified animal models of PBC and analyze their similarities and differences with PBC features in humans.

Keywords: Break of tolerance against PDC-E2, antimitochondrial autoantibodies, autoimmune cholangitis, autoreactive T cells, cytotoxic CD8+ T cells, defective treg cells, cholestasis, AE2 deficiency, deficient bicarbonate secretion.

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Article Details

Year: 2015
Page: [2401 - 2413]
Pages: 13
DOI: 10.2174/1381612821666150316121622
Price: $65

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