Article
Protoporphyrin retention in hepatocytes and Kupffer cells prevents sclerosing cholangitis in erythropoietic protoporphyria mouse model.
Gastroenterology - 1 Oct 2011
Lyoumi Saïd, Abitbol Marie, Rainteau Dominique, Karim Zoubida, Bernex Florence, Oustric Vincent, Millot Sarah, Lettéron Philippe, Heming Nicholas, Guillmot Laurent, Montagutelli Xavier, Berdeaux Gilles, Gouya Laurent, Poupon Raoul, Deybach Jean-Charles, Beaumont Carole, Puy Hervé
Abstract excerpt
BACKGROUND & AIMS: Chronic, progressive hepatobiliary disease is the most severe complication of erythropoietic protoporphyria (EPP) and can require liver transplantation, although the mechanisms that lead to liver failure are unknown. We characterized protoporphyrin-IX (PPIX)-linked hepatobiliary disease in BALB/c and C57BL/6 (Fechm1Pas) mice with mutations in ferrochelatase as models for EPP. METHODS: Fechm1Pas...
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