Mouse Models of Primary Biliary Cirrhosis

Title:Mouse Models of Primary Biliary Cirrhosis

Volume: 21 Issue: 18

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

Affiliation:

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.

Abstract: 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.

Cite this article as:

Concepcion R. Axel and Medina F. Juan, Mouse Models of Primary Biliary Cirrhosis, Current Pharmaceutical Design 2015; 21 (18) . https://dx.doi.org/10.2174/1381612821666150316121622