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Mouse models of primary biliary cirrhosis.
- Source :
-
Current pharmaceutical design [Curr Pharm Des] 2015; Vol. 21 (18), pp. 2401-13. - Publication Year :
- 2015
-
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.
Details
- Language :
- English
- ISSN :
- 1873-4286
- Volume :
- 21
- Issue :
- 18
- Database :
- MEDLINE
- Journal :
- Current pharmaceutical design
- Publication Type :
- Academic Journal
- Accession number :
- 25777756
- Full Text :
- https://doi.org/10.2174/1381612821666150316121622