Detalle Publicación

ARTÍCULO
Approaches to the pathogenesis of primary biliary cirrhosis through animal models
Título de la revista: CLINICS AND RESEARCH IN HEPATOLOGY AND GASTROENTEROLOGY
ISSN: 2210-7401
Volumen: 36
Número: 1
Páginas: 21 - 28
Fecha de publicación: 2012
Resumen:
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 the 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 infiltrating T cells against the inner lipoyl domain in 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 the similarities and differences to PBC features in humans.