Iron and the liver: update 2008.
The cross-talk which has taken place in recent years between clinicians and scientists has resulted in a greater understanding of iron metabolism with the discovery of new iron-related genes including the hepcidin gene which plays a critical role in regulating systemic iron homeostasis. Consequently, the distinction between (a) genetic iron-overload disorders including haemochromatosis related to mutations in the HFE, hemojuvelin, transferrin receptor 2 and hepcidin genes and (b) non-haemochromatotic conditions related to mutations in the ferroportin, ceruloplasmin, transferrin and di-metal transporter 1 genes, and (c) acquired iron-overload syndromes has become easier. However, major challenges still remain which include our understanding of the regulation of hepcidin production, the identification of environmental and genetic modifiers of iron burden and organ damage in iron-overload syndromes, especially HFE haemochromatosis, indications regarding the new oral chelator, deferasirox, and the development of new therapeutic tools interacting with the regulation of iron metabolism.
Deugnier
Yves
Y
Service des maladies du Foie, INSERM CIC 0203, Université de Rennes 1 and IFR 140, CHU Pontchaillou, 35033 Rennes, France. yves.deugnier@univ-rennes1.fr
Brissot
Pierre
P
Loréal
Olivier
O
D023181:Antimicrobial Cationic Peptides; D001565:Benzoates / Q000627:therapeutic use; D000077588:Deferasirox; D006432:Hemochromatosis / Q000188:drug therapy; D064451:Hepcidins; D006706:Homeostasis; D006801:Humans; D007501:Iron / Q000378:metabolism*; D007502:Iron Chelating Agents / Q000627:therapeutic use; D008099:Liver / Q000378:metabolism*; D008107:Liver Diseases / Q000188:drug therapy* / Q000235:genetics*; D014230:Triazoles / Q000627:therapeutic use
D023181:Antimicrobial Cationic Peptides; D001565:Benzoates; C578702:HAMP protein, human; D064451:Hepcidins; D007502:Iron Chelating Agents; D014230:Triazoles; D007501:Iron; D000077588:Deferasirox