Molecular Mechanisms Regulating Hepcidin Revealed by Hepcidin Disorders
Iron is essential for human life, but toxic if present in excess. To avoid iron overload and maintain iron homeostasis, all cells are able to regulate their iron content through the post-transcriptional control of iron genes operated by the cytosolic iron regulatory proteins that interact with iron...
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| Format: | Article |
| Language: | English |
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Wiley
2011-01-01
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| Series: | The Scientific World Journal |
| Online Access: | http://dx.doi.org/10.1100/tsw.2011.130 |
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| author | Clara Camaschella Laura Silvestri |
| author_facet | Clara Camaschella Laura Silvestri |
| author_sort | Clara Camaschella |
| collection | DOAJ |
| description | Iron is essential for human life, but toxic if present in excess. To avoid iron overload and maintain iron homeostasis, all cells are able to regulate their iron content through the post-transcriptional control of iron genes operated by the cytosolic iron regulatory proteins that interact with iron responsive elements on iron gene mRNA. At the systemic level, iron homeostasis is regulated by the liver peptide hepcidin. Disruption of these regulatory loops leads to genetic diseases characterized by iron deficiency (iron-refractory iron-deficiency anemia) or iron overload (hemochromatosis). Alterations of the same systems are also found in acquired disorders, such as iron-loading anemias characterized by ineffective erythropoiesis and anemia of chronic diseases (ACD) associated with common inflammatory conditions. In ACD, iron is present in the body, but maldistributed, being deficient for erythropoiesis, but sequestered in macrophages. Studies of the hepcidin regulation by iron and inflammatory cytokines are revealing new pathways that might become targets of new therapeutic intervention in iron disorders. |
| format | Article |
| id | doaj-art-45de116ef15e48ac9ac4194f90cc92d6 |
| institution | Kabale University |
| issn | 1537-744X |
| language | English |
| publishDate | 2011-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | The Scientific World Journal |
| spelling | doaj-art-45de116ef15e48ac9ac4194f90cc92d62025-02-03T00:59:40ZengWileyThe Scientific World Journal1537-744X2011-01-01111357136610.1100/tsw.2011.130Molecular Mechanisms Regulating Hepcidin Revealed by Hepcidin DisordersClara Camaschella0Laura Silvestri1Vita-Salute University and Division of Genetics and Cell Biology, San Raffaele Scientific Institute, Milan, ItalyVita-Salute University and Division of Genetics and Cell Biology, San Raffaele Scientific Institute, Milan, ItalyIron is essential for human life, but toxic if present in excess. To avoid iron overload and maintain iron homeostasis, all cells are able to regulate their iron content through the post-transcriptional control of iron genes operated by the cytosolic iron regulatory proteins that interact with iron responsive elements on iron gene mRNA. At the systemic level, iron homeostasis is regulated by the liver peptide hepcidin. Disruption of these regulatory loops leads to genetic diseases characterized by iron deficiency (iron-refractory iron-deficiency anemia) or iron overload (hemochromatosis). Alterations of the same systems are also found in acquired disorders, such as iron-loading anemias characterized by ineffective erythropoiesis and anemia of chronic diseases (ACD) associated with common inflammatory conditions. In ACD, iron is present in the body, but maldistributed, being deficient for erythropoiesis, but sequestered in macrophages. Studies of the hepcidin regulation by iron and inflammatory cytokines are revealing new pathways that might become targets of new therapeutic intervention in iron disorders.http://dx.doi.org/10.1100/tsw.2011.130 |
| spellingShingle | Clara Camaschella Laura Silvestri Molecular Mechanisms Regulating Hepcidin Revealed by Hepcidin Disorders The Scientific World Journal |
| title | Molecular Mechanisms Regulating Hepcidin Revealed by Hepcidin Disorders |
| title_full | Molecular Mechanisms Regulating Hepcidin Revealed by Hepcidin Disorders |
| title_fullStr | Molecular Mechanisms Regulating Hepcidin Revealed by Hepcidin Disorders |
| title_full_unstemmed | Molecular Mechanisms Regulating Hepcidin Revealed by Hepcidin Disorders |
| title_short | Molecular Mechanisms Regulating Hepcidin Revealed by Hepcidin Disorders |
| title_sort | molecular mechanisms regulating hepcidin revealed by hepcidin disorders |
| url | http://dx.doi.org/10.1100/tsw.2011.130 |
| work_keys_str_mv | AT claracamaschella molecularmechanismsregulatinghepcidinrevealedbyhepcidindisorders AT laurasilvestri molecularmechanismsregulatinghepcidinrevealedbyhepcidindisorders |