Is HFE involved in increased hepcidin expression and hypoferremia in inflammation and anemia of chronic disease?

Elizabeth A. Milward, Deborah Trinder, Chantelle E.J. Wilcox, Robert S. Britton, Grant A. Ramm, John K. Olynyk – 24 March 2005 – Inflammation influences iron balance in the whole organism. A common clinical manifestation of these changes is anemia of chronic disease (ACD; also called anemia of inflammation). Inflammation reduces duodenal iron absorption and increases macrophage iron retention, resulting in low serum iron concentrations (hyposideremia).

Morphological and biochemical characterization of a human liver in a uPA‐SCID mouse chimera

Philip Meuleman, Louis Libbrecht, Rita De Vos, Bernard de Hemptinne, Kris Gevaert, Joël Vandekerckhove, Tania Roskams, Geert Leroux‐Roels – 24 March 2005 – A small animal model harboring a functional human liver cell xenograft would be a useful tool to study human liver cell biology, drug metabolism, and infections with hepatotropic viruses. Here we describe the repopulation, organization, and function of human hepatocytes in a mouse recipient and the infections with hepatitis B virus (HBV) and hepatitis C virus (HCV) of the transplanted cells.

Two common PFIC2 mutations are associated with the impaired membrane trafficking of BSEP/ABCB11

Hisamitsu Hayashi, Tappei Takada, Hiroshi Suzuki, Hidetaka Akita, Yuichi Sugiyama – 24 March 2005 – Progressive familial intrahepatic cholestasis type 2 (PFIC2) is caused by a mutation in the bile salt export pump (BSEP/ABCB11) gene. However, the mechanisms for the deficiency in the function of two mutations (E297G and D482G), which are frequently found in European patients, have not yet been identified. In the present study, we examined the transport activity and cellular localization of these two mutants in human embryonic kidney 293 and Madin‐Darby canine kidney II cells, respectively.

Abnormal concentrations of esterified carnitine in bile: A feature of pediatric acute liver failure with poor prognosis

Benjamin L. Shneider, Piero Rinaldo, Sukru Emre, John Bucuvalas, Robert Squires, Michael Narkewicz, Gabriel Gondolesi, Margret Magid, Raffaella Morotti, Linda S. Hynan – 24 March 2005 – The etiology of acute liver failure in children is unknown in a large number of cases. Defects in fatty acid oxidation have been shown to lead to severe liver injury. This retrospective analysis examined the bile acylcarnitine profiles of 27 children with acute liver failure who underwent liver transplantation or died.

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