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Genomes and Genes | Elizabeta NemethSummaryAffiliation: University of California Country: USA Publications
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Publications
Regulation of iron metabolism by hepcidinElizabeta Nemeth
Departments of Medicine and Pathology, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California 90095, USA
Annu Rev Nutr 26:323-42. 2006..The emergence of hepcidin as the pathogenic factor in most systemic iron disorders should provide important opportunities for improving their diagnosis and treatment...- Iron regulation and erythropoiesisElizabeta Nemeth
David Geffen School of Medicine, University of California, Los Angeles, California 90095, USA
Curr Opin Hematol 15:169-75. 2008..Major advances have been made in understanding the regulation of hepcidin production, and consequently the availability of iron for erythropoiesis... - Hepcidin in beta-thalassemiaElizabeta Nemeth
Department of Medicine, University of California at Los Angeles, Los Angeles, CA 90095, USA
Ann N Y Acad Sci 1202:31-5. 2010..Hepcidin diagnostics and future therapeutic agonists may help in management of patients with beta-thalassemia... - Hepcidin biology and therapeutic applicationsElizabeta Nemeth
David Geffen School of Medicine, UCLA, 10833 LeConte Ave, CHS 37 131, Los Angeles, CA 90095, USA
Expert Rev Hematol 3:153-5. 2010..The session included three 30-min presentations... - The role of hepcidin in iron metabolismElizabeta Nemeth
David Geffen School of Medicine, University of California, Los Angeles, California 90095, USA
Acta Haematol 122:78-86. 2009..Diagnostic and therapeutic applications of this new knowledge are beginning to emerge. Dr. Ernest Beutler played a significant role in advancing our understanding of the function of hepcidin. This review is dedicated to his memory... - Reduction of serum hepcidin by hemodialysis in pediatric and adult patientsJoshua Zaritsky
Department of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, California, USA
Clin J Am Soc Nephrol 5:1010-4. 2010.... - Minihepcidins are rationally designed small peptides that mimic hepcidin activity in mice and may be useful for the treatment of iron overloadGloria C Preza
Department of Pathology, UCLA, Los Angeles, California, USA
J Clin Invest 121:4880-8. 2011..Moreover, liver iron concentrations were lower in mice chronically treated with minihepcidins than those in mice treated with solvent alone. Minihepcidins may be useful for the treatment of iron overload disorders... - Iron sequestration and anemia of inflammationTomas Ganz
Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA, USA
Semin Hematol 46:387-93. 2009..The supply of iron to hemoglobin synthesis becomes limiting, eventually resulting in anemia. Depending on the details of the underlying disease, other inflammation-related mechanisms may also contribute to anemia... - Iron transferrin regulates hepcidin synthesis in primary hepatocyte culture through hemojuvelin and BMP2/4Lan Lin
Departments of Pathology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095 1690, USA
Blood 110:2182-9. 2007..Fresh primary hepatocytes constitute a sufficient system for the regulation of hepcidin by physiologic iron stimuli and will greatly facilitate studies of major disorders of iron homeostasis... - Synthetic hepcidin causes rapid dose-dependent hypoferremia and is concentrated in ferroportin-containing organsSeth Rivera
UCLA Department of Medicine, 10833 Le Conte Ave, CHS 37 131, Los Angeles, CA 90095, USA
Blood 106:2196-9. 2005..The rapid and sustained action of a single dose of hepcidin makes it an appealing agent for the prevention of iron accumulation in hereditary hemochromatosis... - Evidence for distinct pathways of hepcidin regulation by acute and chronic iron loading in miceEmilio Ramos
Chemistry and Biochemistry, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA
Hepatology 53:1333-41. 2011..A distinct regulatory mechanism that senses hepatic iron may modulate hepcidin response to chronic iron loading... - The molecular basis of hepcidin-resistant hereditary hemochromatosisAugustine Fernandes
Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA
Blood 114:437-43. 2009..Our observations explain why the mutations in C326 Fpn residue produce a severe form of hemochromatosis with iron overload at an early age... - IL-6 mediates hypoferremia of inflammation by inducing the synthesis of the iron regulatory hormone hepcidinElizabeta Nemeth
Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles UCLA, Los Angeles, California, USA
J Clin Invest 113:1271-6. 2004.... - Hepcidin and disorders of iron metabolismTomas Ganz
Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, California 90095, USA
Annu Rev Med 62:347-60. 2011..The hepcidin-ferroportin axis is the principal regulator of extracellular iron homeostasis in health and disease, and is a promising target for the diagnosis and treatment of iron disorders and anemias... - Involvement of hepcidin in the anemia of multiple myelomaSanjai Sharma
West Los Angeles VA University of California Los Angeles Medical Center, Los Angeles, CA 90073, USA
Clin Cancer Res 14:3262-7. 2008..Because interleukin (IL)-6 is a potent inducer of hepcidin expression and its levels are elevated in multiple myeloma, we studied the role of hepcidin in the anemia of multiple myeloma... - Hepcidin--a potential novel biomarker for iron status in chronic kidney diseaseJoshua Zaritsky
Department of Pediatrics, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California, USA
Clin J Am Soc Nephrol 4:1051-6. 2009..Hepcidin is a key regulator of iron homeostasis, but its study in the setting of chronic kidney disease (CKD) has been hampered by the lack of validated serum assays... - Soluble hemojuvelin is released by proprotein convertase-mediated cleavage at a conserved polybasic RNRR siteLan Lin
Department of Pathology, David Geffen School of Medicine, University of California, Los Angeles UCLA, Los Angeles, CA 90095, USA
Blood Cells Mol Dis 40:122-31. 2008..In this report, we investigate the proteinase involved in the release of s-hemojuvelin and show that s-hemojuvelin is released by a proprotein convertase through the cleavage at a conserved polybasic RNRR site... - Regulation of iron acquisition and iron distribution in mammalsTomas Ganz
Department of Medicine and Pathology, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA 90095 1690, USA
Biochim Biophys Acta 1763:690-9. 2006..Deficiency of hepcidin is the unifying cause of hereditary hemochromatoses, and excessive cytokine-stimulated hepcidin production causes hypoferremia and contributes to anemia of inflammation... - Hepcidin regulates cellular iron efflux by binding to ferroportin and inducing its internalizationElizabeta Nemeth
Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA, USA
Science 306:2090-3. 2004..The posttranslational regulation of ferroportin by hepcidin may thus complete a homeostatic loop: Iron regulates the secretion of hepcidin, which in turn controls the concentration of ferroportin on the cell surface... - Hepcidin excess induces the sequestration of iron and exacerbates tumor-associated anemiaSeth Rivera
Department of Medicine, David Geffen School of Medicine at the University of California, Los Angeles, CA 90095, USA
Blood 105:1797-802. 2005..Hepcidin contributes to AI by shunting iron away from erythropoiesis and sequestering it in the liver, predominantly in hepatocytes... - The N-terminus of hepcidin is essential for its interaction with ferroportin: structure-function studyElizabeta Nemeth
Department of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095 1690, USA
Blood 107:328-33. 2006..G71D and K83R, substitutions previously described in humans, did not affect hepcidin activity. Apart from the essential nature of the N-terminus, hepcidin structure appears permissive for mutations... - Iron imports. IV. Hepcidin and regulation of body iron metabolismTomas Ganz
Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095 1690, USA
Am J Physiol Gastrointest Liver Physiol 290:G199-203. 2006..Hepcidin deficiency due to the dysregulation of its synthesis causes most known forms of hemochromatosis... - The hepcidin-ferroportin system as a therapeutic target in anemias and iron overload disordersTomas Ganz
Department of Pulmonary and Critical Care Medicine, University of California Los Angeles, Los Angeles, CA, USA
Hematology Am Soc Hematol Educ Program 2011:538-42. 2011.... - Hepcidin, a putative mediator of anemia of inflammation, is a type II acute-phase proteinElizabeta Nemeth
Department of Medicine, David Geffen School of Medicine, and the West Los Angeles Veterans Administration Hospital, University of California, Los Angeles 90095, USA
Blood 101:2461-3. 2003..The linkage of hepcidin induction to inflammation in humans supports its proposed role as a key mediator of anemia of inflammation... - Immunoassay for human serum hepcidinTomas Ganz
Intrinsic LifeSciences LLC, La Jolla, CA, USA
Blood 112:4292-7. 2008..The new serum hepcidin enzyme-linked immunosorbent assay yields accurate and reproducible measurements that appropriately reflect physiologic, pathologic, and genetic influences, and is informative about the etiology of iron disorders... - Hepcidin is decreased in TFR2 hemochromatosisElizabeta Nemeth
Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA
Blood 105:1803-6. 2005..The only 2 cases with normal hepcidin values had concomitant inflammatory conditions. Our data indicate that TFR2 is a modulator of hepcidin production in response to iron... - The hepcidin-binding site on ferroportin is evolutionarily conservedIvana De Domenico
Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
Cell Metab 8:146-56. 2008..The affinity of hepcidin for the HBD permits a rapid, sensitive assay of hepcidin from all species and yields insights into the evolution of hepcidin... - Primary iron overload with inappropriate hepcidin expression in V162del ferroportin diseaseHeinz Zoller
Clinical Division of Gastroenterology and Hepatology, Innsbruck Medical University, Innsbruck, Austria
Hepatology 42:466-72. 2005..Finally, macrophage iron storage in ferroportin disease is associated with elevated serum pro-hepcidin levels... - The molecular basis of ferroportin-linked hemochromatosisIvana De Domenico
Dipartimento di Scienze Microbiologiche Genetiche e Molecolari, Universita di Messina, 98166 Messina, Italy
Proc Natl Acad Sci U S A 102:8955-60. 2005..The behavior of mutant Fpn in cell culture and the ability of mutant Fpn to act as a dominant negative explain the dominant inheritance of the disease as well as the different patient phenotypes... - Time-course analysis of hepcidin, serum iron, and plasma cytokine levels in humans injected with LPSErwin Kemna
Department of Clinical Chemistry 564, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands
Blood 106:1864-6. 2005..These in vivo human results confirm the importance of the IL-6-hepcidin axis in the development of hypoferremia in inflammation and highlight the rapid responsiveness of this iron regulatory system... - DMT1 mutation: response of anemia to darbepoetin administration and implications for iron homeostasisDagmar Pospisilova
Blood 108:404-5. 2006 - Hepcidin and iron-related gene expression in subjects with Dysmetabolic Hepatic Iron OverloadDonatella Barisani
Department of Experimental Medicine, University of Milano Bicocca, Via Cadore 48, Monza 20052, Milan, Italy
J Hepatol 49:123-33. 2008..Since the pathophysiology of Dysmetabolic Hepatic Iron Overload (DHIO) is still obscure, the aim of this study was to evaluate, in these patients, possible alterations in iron-related molecule expression... - Hepcidin and iron-loading anemiasElizabeta Nemeth
Haematologica 91:727-32. 2006 - The molecular mechanism of hepcidin-mediated ferroportin down-regulationIvana De Domenico
Department of Pathology, School of Medicine, University of Utah, Salt Lake City, UT 84132, USA
Mol Biol Cell 18:2569-78. 2007..Depletion of proteins involved in multivesicular body trafficking (Endosome Sorting Complex Required for Transport proteins), by small-interfering RNA, reduces the trafficking of Fpn-green fluorescent to the lysosome... - Effects of plasma transfusion on hepcidin production in human congenital hypotransferrinemiaPaola Trombini
Department of Clinical Medicine and Centre for Diagnosis and Therapy of Hemochromatosis, University of Milano Bicocca, San Gerardo Hospital, Monza, Milan, Italy
Haematologica 92:1407-10. 2007..Time-course of urinary hepcidin and serum transferrin concentration suggests that hepcidin production is regulated by the combination of marrow iron requirements and iron supply by transferrin... - Blunted hepcidin response to oral iron challenge in HFE-related hemochromatosisAlberto Piperno
Department of Clinical Medicine and Prevention, Milano Bicocca University, San Gerardo Hospital, Monza, Italy
Blood 110:4096-100. 2007..The hepcidin response to oral iron is blunted in HFE-related hemochromatosis and not improved after iron depletion. The findings support the involvement of HFE in iron sensing and subsequent regulation of hepcidin... - Liver iron concentrations and urinary hepcidin in beta-thalassemiaRaffaella Origa
Dipartimento di Scienze Biomediche e Biotecnologie, Universita di Cagliari, Ospedale Microcitemico ASL8 Cagliari, Italy
Haematologica 92:583-8. 2007..Hepcidin deficiency is the cause of iron overload in most forms of hereditary hemochromatosis. We sought to determine hepcidin's role in the pathogenesis of iron overload in b-thalassemia... - Mutations in HFE2 cause iron overload in chromosome 1q-linked juvenile hemochromatosisGeorge Papanikolaou
First Department of Internal Medicine, National and Kapodistrian University of Athens, School of Medicine, Laikon General Hospital, Athens 11527, Greece
Nat Genet 36:77-82. 2004..Urinary hepcidin levels were depressed in individuals with juvenile hemochromatosis, suggesting that hemojuvelin is probably not the hepcidin receptor. Rather, HFE2 seems to modulate hepcidin expression... - Molecular and clinical correlates in iron overload associated with mutations in ferroportinIvana De Domenico
Dipartimento di Scienze Microbiologiche Genetiche e Molecolari, Universita di Messina, 98166 Messina, Italia
Haematologica 91:1092-5. 2006..We suggest that FpnG80S represents a class of Fpn mutants whose behavior in vitro does not explain the patients' phenotype... - Impaired intestinal iron absorption in Crohn's disease correlates with disease activity and markers of inflammationGaith Semrin
Center for Inflammatory Bowel Disease and Divisions of Gastroenterology and Nutrition, Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA
Inflamm Bowel Dis 12:1101-6. 2006..These findings suggest that oral iron may be of limited benefit to these patients. Future study is needed to define the molecular basis for impaired iron absorption... - Hepcidin in iron overload disordersGeorge Papanikolaou
First Department of Medicine, National and Kapodistrian University of Athens, Greece
Blood 105:4103-5. 2005..These findings extend the spectrum of iron disorders with hepcidin deficiency and underscore the critical importance of the hepcidin-ferroportin interaction in iron homeostasis... - Hepcidin levels in humans are correlated with hepatic iron stores, hemoglobin levels, and hepatic functionLenaick Detivaud
Institut National de la Sante et de la Recherche Medicale, Rennes, France
Blood 106:746-8. 2005..Moreover, we found in humans that hepcidin levels correlated with hepatic iron stores and hemoglobin levels and may also be affected by hepatic dysfunction... - Urinary hepcidin in congenital chronic anemiasSusan L Kearney
Division of Hematology Oncology, Children s Hospital, Boston, Massachusetts, USA
Pediatr Blood Cancer 48:57-63. 2007..Hepcidin, a regulator for iron homeostasis, is induced by inflammation and iron burden and suppressed by anemia and hypoxia. This study was conducted to determine the hepcidin levels in patients with congenital chronic anemias... - Measurement of urinary hepcidin levels by SELDI-TOF-MS in HFE-hemochromatosisClaudia Bozzini
Department of Clinical and Experimental Medicine, University of Verona, Policlinico G B Rossi, 37134 Verona, Italy
Blood Cells Mol Dis 40:347-52. 2008..This study measured urinary hepcidin levels by SELDI-TOF-MS in a large group of HH patients at diagnosis and during treatment, including both C282Y homozygous and C282Y/H63D compound heterozygotes... - Novel urine hepcidin assay by mass spectrometryErwin Kemna
Department of Clinical Chemistry 564, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500 HB Nijmegen, The Netherlands
Blood 106:3268-70. 2005..In conclusion, this novel MS urine hepcidin assay is easy to perform and available to a wide audience. This enables the implementation of hepcidin measurements in large clinical studies...
Research Grants
- REGULATION OF HEPCIDIN BY IRON AND OXYGENElizabeta Nemeth; Fiscal Year: 2007....
- The Hepcidin-Ferroportin Axis in Anemia of Inflammation: Mechanisms and TargetsElizabeta Nemeth; Fiscal Year: 2010..PUBLIS HEALTH RELEVANCE: The proposed project will help understand and treat a common form of anemia which develops in infections and inflammatory disorders. ..