Molecular mechanisms regulating hepcidin revealed by hepcidin disorders

Clara Camaschella, Laura Silvestri

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish
Pages (from-to)1357-1366
Number of pages10
JournalTheScientificWorldJournal
Volume11
DOIs
Publication statusPublished - Jul 7 2011

Fingerprint

Hepcidins
Iron
iron
anemia
Anemia
Iron Overload
Erythropoiesis
Homeostasis
Chronic Disease
homeostasis
Iron-Regulatory Proteins
Inborn Genetic Diseases
Hemochromatosis
Genes
Poisons
Macrophages

Keywords

  • Anemia
  • Erythropoiesis
  • Hepcidin
  • Iron
  • Iron metabolism

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)
  • Environmental Science(all)
  • Medicine(all)

Cite this

Molecular mechanisms regulating hepcidin revealed by hepcidin disorders. / Camaschella, Clara; Silvestri, Laura.

In: TheScientificWorldJournal, Vol. 11, 07.07.2011, p. 1357-1366.

Research output: Contribution to journalArticle

Camaschella, Clara ; Silvestri, Laura. / Molecular mechanisms regulating hepcidin revealed by hepcidin disorders. In: TheScientificWorldJournal. 2011 ; Vol. 11. pp. 1357-1366.
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