Protoporphyrin retention in hepatocytes and Kupffer cells prevents sclerosing cholangitis in erythropoietic protoporphyria mouse model.

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

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.

Affiliation

Lyoumi S; INSERM U773, Centre de Recherche Biomédicale Bichat Beaujon CRB3, Université Paris Diderot, site Bichat, Centre de reference des maladies inflammatoires des voies biliaires, service d'Hépatologie-Gastroentérologie, Hôpital Saint Antoine, Paris, France.

Gastroenterology ; 141(4): 1509-19, 1519.e1-3, 2011 Oct.

Article in En | MEDLINE | ID: mdl-21762662

Subject(s)

Cholangitis, Sclerosing/prevention & control; Hepatocytes/metabolism; Kupffer Cells/metabolism; Porphyria, Erythropoietic/metabolism; Protoporphyrins/metabolism; Animals; Bile Acids and Salts/metabolism; Cholangitis, Sclerosing/genetics; Cholangitis, Sclerosing/metabolism; Cholangitis, Sclerosing/pathology; Cholesterol/metabolism; Disease Models, Animal; Ferrochelatase/genetics; Ferrochelatase/metabolism; Gene Expression Regulation; Genotype; Hepatocytes/pathology; Kupffer Cells/pathology; Lipoprotein-X/blood; Liver Cirrhosis/metabolism; Liver Cirrhosis/prevention & control; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Phenotype; Phospholipids/metabolism; Point Mutation; Porphyria, Erythropoietic/complications; Porphyria, Erythropoietic/genetics; Porphyria, Erythropoietic/pathology; Severity of Illness Index

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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Protoporphyrins / Cholangitis, Sclerosing / Porphyria, Erythropoietic / Hepatocytes / Kupffer Cells Limits: Animals Language: En Journal: Gastroenterology Year: 2011 Type: Article Affiliation country: France

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