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SHR Neuro Krebs Kardio Lipid

Trauner, M; Fickert, P; Baghdasaryan, A; Claudel, T; Halilbasic, E; Moustafa, T; Wagner, M; Zollner, G.
New insights into autoimmune cholangitis through animal models.
Dig Dis. 2010; 28(1):99-104
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Autor/innen der Med Uni Graz:
Baghdasaryan Anna
Claudel Thierry
Fickert Peter
Halilbasic Emina
Moustafa Tarek
Trauner Michael
Wagner Martin
Zollner Gernot
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Abstract:
Improving our understanding of the pathogenesis of chronic immune-mediated cholangiopathies such as primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), as well as the development of novel diagnostic, prognostic and therapeutic tools for these disorders critically depends on easily reproducible animal models. Recently, several spontaneous mouse models for PBC (not requiring previous manipulations for breakdown of immunotolerance) have been reported, including NOD.c3c4 and NOD.c3c4-derived mice, IL-2Ralpha(-/-) mice, dominant negative TGF-beta receptor II mice and Ae2(a,b)(-/-) mice. To date, no animal model exhibits all of the attributes of PSC. Rodent models induced by bacterial cell components or colitis may help to explain the strong association between PSC and inflammatory bowel disease. Other models include direct injury to biliary epithelia, peribiliary vascular endothelia or portal venous endothelia. Mice with targeted disruption of the Mdr2 (Abcb4) gene encoding a canalicular phospholipid flippase (Mdr2(-/-) mice) spontaneously develop sclerosing cholangitis with macroscopic and microscopic features of human PSC. Another example for a transporter involved in the pathogenesis of sclerosing cholangitis is the cystic fibrosis transmembrane conductance regulator (CFTR/ABCC7). Xenobiotics and drugs may also lead to bile duct injury and biliary fibrosis via direct toxic and indirect immune-mediated injury. Hydrophobic bile acids, such as lithocholic acid, cause bile duct injury and destructive cholangitis with periductal fibrosis resembling sclerosing cholangitis. These models have enhanced our understanding of the pathogenesis of PBC and PSC and will hopefully result in improved treatment of these disorders. Copyright 2010 S. Karger AG, Basel.
Find related publications in this database (using NLM MeSH Indexing)
Animals -
Autoimmune Diseases - immunology
Cholangitis - immunology
Cholangitis, Sclerosing - immunology Cholangitis, Sclerosing - physiopathology Cholangitis, Sclerosing - therapy
Disease Models, Animal -
Humans -
Liver Cirrhosis, Biliary - immunology Liver Cirrhosis, Biliary - physiopathology
Mice -
Mice, Knockout -

Find related publications in this database (Keywords)
Primary biliary cirrhosis
Primary sclerosing cholangitis
Animal models
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