Analysis of Liver Repair Mechanisms in Alagille Syndrome and Biliary Atresia Reveals a Role for Notch Signaling

Patients with Alagille syndrome (AGS), a genetic disorder of Notch signaling, suffer from severe ductopenia and cholestasis, but progression to biliary cirrhosis is rare. Instead, in biliary atresia (BA) severe cholestasis is associated with a pronounced “ductular reaction” and rapid progression to...

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Autores principales: Fabris, Luca, Cadamuro, Massimiliano, Guido, Maria, Spirli, Carlo, Fiorotto, Romina, Colledan, Michele, Torre, Giuliano, Alberti, Daniele, Sonzogni, Aurelio, Okolicsanyi, Lajos, Strazzabosco, Mario
Formato: Artigo
Lenguaje:Inglês
Publicado: American Society for Investigative Pathology 2007
Materias:
Regular Articles
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC1934520/
https://ncbi.nlm.nih.gov/pubmed/17600123
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.2353/ajpath.2007.070073
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