Journal ArticleDOI
Disorders of iron metabolism.
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
Hephaestin, a ceruloplasmin homologue implicated in intestinal iron transport, is defective in the sla mouse.
Chris D. Vulpe,Yien-Ming Kuo,T. L. Murphy,L. Cowley,Candice C. Askwith,Natasha Libina,Jane Gitschier,Gregory J. Anderson +7 more
TL;DR: It is suggested that the hephaestin protein is a multi–copper ferroxidase necessary for iron egress from intestinal enterocytes into the circulation and that it is an important link between copper and iron metabolism in mammals.
Journal ArticleDOI
Nramp2 is mutated in the anemic Belgrade (b) rat: Evidence of a role for Nramp2 in endosomal iron transport
Mark D. Fleming,Michelle A. Romano,Maureen A. Su,Laura M. Garrick,Michael D. Garrick,Nancy C. Andrews +5 more
TL;DR: The hypothesis that Nramp2 is the protein defective in the Belgrade rat is confirmed and the possibility that the phenotype shared by mk and b animals is unique to the G185R mutation raised, as the phenotypic characteristics of these animals indicate that NRamp2 are essential both for normal intestinal iron absorption and for transport of iron out of the transferrin cycle endosome.
Journal ArticleDOI
The hemochromatosis gene product complexes with the transferrin receptor and lowers its affinity for ligand binding
John N. Feder,David Penny,Alivelu Irrinki,Vince K. Lee,José A. Lebrón,Nicole Watson,Zenta Tsuchihashi,Elliott Sigal,Pamela J. Bjorkman,Randall C. Schatzman +9 more
TL;DR: A molecular link between HFE and a key protein involved in iron transport, the TfR, is established and the possibility that alterations in this regulatory mechanism may play a role in the pathogenesis of hereditary hemochromatosis is raised.
Journal ArticleDOI
A Population-Based Study of the Clinical Expression of the Hemochromatosis Gene
TL;DR: Eight of the 16 homozygous subjects had clinical findings that were consistent with the presence of hereditary hemochromatosis, such as hepatomegaly, skin pigmentation, and arthritis, in a population of white adults of northern European ancestry.
Journal ArticleDOI
The β-Thalassemias
TL;DR: Thalassemia is a disease of Mediterranean origin occurring in children of Italian origin and associated with splenomegaly and characteristic bone changes not confined to the Mediterranean, but occurring widely.
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