Document Detail

Regulation of hepcidin and iron-overload disease.
MedLine Citation:
PMID:  19400694     Owner:  NLM     Status:  MEDLINE    
Hepcidin, a 25-amino-acid antimicrobial peptide, is the central regulator of iron homeostasis. Hepcidin transcription is upregulated by inflammatory cytokines, iron, and bone morphogenetic proteins and is downregulated by iron deficiency, ineffective erythropoiesis, and hypoxia. The iron transporter ferroportin is the cognate receptor of hepcidin and is destroyed as a result of interaction with the peptide. Except for inherited defects of ferroportin and hepcidin itself, all forms of iron-storage disease appear to arise from hepcidin dysregulation. Studies using multiple approaches have begun to delineate the molecular mechanisms that regulate hepcidin expression, particularly at the transcriptional level. Knowledge of the regulation of hepcidin by inflammation, iron, erythropoiesis, and hypoxia will lead to an understanding of the pathogenesis of primary hemochromatosis, secondary iron overload, and anemia of inflammatory disease.
Pauline L Lee; Ernest Beutler
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Annual review of pathology     Volume:  4     ISSN:  1553-4014     ISO Abbreviation:  Annu Rev Pathol     Publication Date:  2009  
Date Detail:
Created Date:  2009-04-29     Completed Date:  2009-05-28     Revised Date:  2010-04-29    
Medline Journal Info:
Nlm Unique ID:  101275111     Medline TA:  Annu Rev Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  489-515     Citation Subset:  IM    
Department of Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, CA 92037, USA.
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MeSH Terms
Anemia / metabolism
Antimicrobial Cationic Peptides / chemistry,  genetics,  metabolism*
Cation Transport Proteins / metabolism
Hemochromatosis / metabolism
Iron / metabolism*
Iron Overload / genetics,  metabolism*
Membrane Proteins / metabolism
Protein Conformation
Structure-Activity Relationship
Transcription, Genetic
Grant Support
Reg. No./Substance:
0/Antimicrobial Cationic Peptides; 0/Cation Transport Proteins; 0/HFE2 protein, human; 0/Membrane Proteins; 0/hepcidin; 0/metal transporting protein 1; 7439-89-6/Iron

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