Experimental evidence on the immunopathogenesis of primary biliary cirrhosis.
Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease for which an autoimmune pathogenesis is supported by clinical and experimental data, including the presence of autoantibodies and autoreactive T cells. The etiology remains to be determined, yet data suggest that both a susceptible genetic background and unknown environmental factors determine disease onset. Multiple infectious and chemical candidates have been proposed to trigger the disease in a genetically susceptible host, mostly by molecular mimicry. Most recently, several murine models have been reported, including genetically determined models as well as models induced by immunization with xenobiotics and bacteria.
Selmi
Carlo
C
Department of Translational Medicine, Università degli Studi di Milano, Milan, Italy. carlo.selmi@unimi.it
Meda
Francesca
F
Kasangian
Anaid
A
Invernizzi
Pietro
P
Tian
Zhigang
Z
Lian
Zhexiong
Z
Podda
Mauro
M
Gershwin
M Eric
ME
D000818:Animals; D001327:Autoimmune Diseases / Q000276:immunology; D004195:Disease Models, Animal; D020022:Genetic Predisposition to Disease; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000139:chemically induced / Q000235:genetics / Q000276:immunology* / Q000382:microbiology; D015262:Xenobiotics / Q000009:adverse effects
D015262:Xenobiotics