The iron driven pathway of hepcidin synthesis.
Iron is an essential trace element in mammalian metabolism. Body iron stores require a tight regulation to avoid detrimental effects due to iron excess or to iron deficiency. Iron losses being not adaptable, iron balance is controlled only through intestinal iron absorption which is regulated by the hepatic peptide hepcidin. Hepcidin synthesis is controlled by several genes including the HFE, hemojuvelin and transferrin receptor 2 genes. Mutations in these genes lead to a phenotype of hemochromatosis. Recently, the bone morphogenetic protein 6 was shown to be the key endogenous ligand involved in the cascade regulating hepcidin synthesis.
Inserm U 991, CIC 0203, CHU, université de Rennes 1, Rennes, France. yves.deugnier@univ-rennes1.fr
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Deugnier
D023181:Antimicrobial Cationic Peptides / Q000096:biosynthesis* / Q000502:physiology; D055418:Bone Morphogenetic Protein 6 / Q000502:physiology*; D058851:GPI-Linked Proteins / Q000235:genetics; D006432:Hemochromatosis / Q000235:genetics; D000071020:Hemochromatosis Protein; D064451:Hepcidins; D015395:Histocompatibility Antigens Class I / Q000235:genetics; D006801:Humans; D007501:Iron / Q000378:metabolism*; D008099:Liver / Q000378:metabolism*; D008565:Membrane Proteins / Q000235:genetics; D009154:Mutation; D011990:Receptors, Transferrin / Q000235:genetics
D023181:Antimicrobial Cationic Peptides; D055418:Bone Morphogenetic Protein 6; D058851:GPI-Linked Proteins; C578702:HAMP protein, human; C103673:HFE protein, human; C481006:HJV protein, human; D000071020:Hemochromatosis Protein; D064451:Hepcidins; D015395:Histocompatibility Antigens Class I; D008565:Membrane Proteins; D011990:Receptors, Transferrin; C503640:TFR2 protein, human; D007501:Iron