Journal ArticleDOI
Disorders of iron metabolism.
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TLDR
Iron has the capacity to accept and donate electrons readily, interconverting between ferric (Fe2+) and ferrous (Fe3+) forms, which makes it a useful component of cytochromes, oxygen-binding molecules, and many enzymes.Abstract:
Iron has the capacity to accept and donate electrons readily, interconverting between ferric (Fe2+) and ferrous (Fe3+) forms. This capability makes it a useful component of cytochromes, oxygen-bind...read more
Citations
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Journal ArticleDOI
IL-6 mediates hypoferremia of inflammation by inducing the synthesis of the iron regulatory hormone hepcidin
Elizabeta Nemeth,Seth Rivera,Victoria Gabayan,Charlotte Keller,Sarah Taudorf,Bente Klarlund Pedersen,Tomas Ganz +6 more
TL;DR: These studies in human liver cell cultures, mice, and human volunteers indicate that IL-6 is the necessary and sufficient cytokine for the induction of hepcidin during inflammation and that the IL- 6-hepcid in axis is responsible for the hypoferremia of inflammation.
Journal ArticleDOI
Positional cloning of zebrafish ferroportin1 identifies a conserved vertebrate iron exporter
Adriana Donovan,Alison Brownlie,Alison Brownlie,Yi Zhou,Jennifer L. Shepard,Stephen J. Pratt,John Moynihan,Barry H. Paw,Anna Drejer,Bruce A. Barut,Bruce A. Barut,Agustín G. Zapata,Terence C. Law,Carlo Brugnara,Samuel E. Lux,Geraldine S. Pinkus,Jack L. Pinkus,Paul D. Kingsley,James Palis,Mark D. Fleming,Mark D. Fleming,Nancy C. Andrews,Nancy C. Andrews,Leonard I. Zon,Leonard I. Zon +24 more
TL;DR: The gene responsible for the hypochromic anaemia of the zebrafish mutant weissherbst is identified and Ferroportin1 function may be perturbed in mammalian disorders of iron deficiency or overload.
Journal ArticleDOI
Hepcidin, a key regulator of iron metabolism and mediator of anemia of inflammation.
TL;DR: Human hepcidin, a 25-amino acid peptide made by hepatocytes, may be a new mediator of innate immunity and the long-sought iron-regulatory hormone and its role in iron metabolism could lead to new therapies for hemochromatosis and anemia of inflammation.
Journal ArticleDOI
Targeted Drug Delivery via the Transferrin Receptor-Mediated Endocytosis Pathway
TL;DR: The OX26 monoclonal antibody against the rat transferrin receptor offers great promise in the delivery of therapeutic agents across the blood-brain barrier to the brain and serves as a potential alternative to viral vector for gene therapy.
Journal ArticleDOI
Hepcidin and iron homeostasis.
Tomas Ganz,Elizabeta Nemeth +1 more
TL;DR: Hepcidin, ferroportin and their regulators represent potential targets for the diagnosis and treatment of iron disorders and anemias.
References
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Journal ArticleDOI
Microcytic anemia with iron malabsorption: An inherited disorder of iron metabolism
Kip R. Hartman,James A. Barker +1 more
TL;DR: Two siblings were identified with severe hypoproliferative microcytic anemia and iron malabsorption, in the absence of any gastrointestinal disorder or blood loss, that appears to be a rare form of inherited anemia characterized by iron mal absorption and disordered iron metabolism that only partially corrects after the administration of parenteral iron.
Journal ArticleDOI
Inherited HFE-unrelated hemochromatosis in Italian families.
Clara Camaschella,Silvia Fargion,Maurizio Sampietro,Antonella Roetto,Sandra Bosio,Giovanni Garozzo,Cristina Arosio,Alberto Piperno +7 more
TL;DR: The results point to genetic forms of hemochromatosis not associated with HFE and raise the problem of whether non‐HFE hemochROMatosis in Italy is related to the “juvenile” form, and emphasize the importance of phenotypic as well as genetic diagnosis of HH.
Journal ArticleDOI
Malabsorption and defective utilization of iron in three siblings
TL;DR: Three siblings with iron deficiency anemia without evidence of reduced iron intake or gastrointestinal blood loss appear to have a familial disorder characterized by impaired iron absorption and utilization, similar to that observed in the mk/mk mouse.
Journal ArticleDOI
Is there a link between African iron overload and the described mutations of the hereditary haemochromatosis gene
TL;DR: PCR analysis of DNA from 25 southern Africans identified by segregation analysis as having a high probability of carrying the putative African iron‐loading gene failed to identify any subjects with the C282Y mutation, suggesting a possible genetic defect in African iron overload.
Journal ArticleDOI
Idiopathic neonatal iron storage involving the liver, pancreas, heart, and endocrine and exocrine glands
Sidney Goldfischer,Sidney Goldfischer,Sidney Goldfischer,Harold W. Grotsky,Harold W. Grotsky,Harold W. Grotsky,Chung-Ho Chang,Chung-Ho Chang,Chung-Ho Chang,Errol L. Berman,Errol L. Berman,Errol L. Berman,Robert R. Richert,Robert R. Richert,Robert R. Richert,Sushama D. Karmarkar,Sushama D. Karmarkar,Sushama D. Karmarkar,Jacqueline O. Roskamp,Jacqueline O. Roskamp,Jacqueline O. Roskamp,Rachel Morecki,Rachel Morecki,Rachel Morecki +23 more
TL;DR: Neonatal iron storage disease is clinically and pathologically distinct from Zellweger's cerebrohepatorenal syndrome and hypermethioninemia (tyrosinemia) neonatal diseases in which large stores of iron are present in hepatocytes.
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