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铁调素在铁代谢中的作用。

The role of hepcidin in iron metabolism.

作者信息

Nemeth Elizabeta, Ganz Tomas

机构信息

David Geffen School of Medicine, University of California, Los Angeles, California 90095, USA.

出版信息

Acta Haematol. 2009;122(2-3):78-86. doi: 10.1159/000243791. Epub 2009 Nov 10.

DOI:10.1159/000243791
PMID:19907144
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2855274/
Abstract

Hepcidin is the central regulator of systemic iron homeostasis. Dysregulation of hepcidin production results in a variety of iron disorders. Hepcidin deficiency is the cause of iron overload in hereditary hemochromatosis, iron-loading anemias, and hepatitis C. Hepcidin excess is associated with anemia of inflammation, chronic kidney disease and iron-refractory iron deficiency anemia. 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.

摘要

铁调素是全身铁稳态的核心调节因子。铁调素产生的失调会导致多种铁紊乱。铁调素缺乏是遗传性血色素沉着症、铁过载性贫血和丙型肝炎中铁过载的原因。铁调素过量与炎症性贫血、慢性肾病和铁难治性缺铁性贫血有关。这一新知识的诊断和治疗应用已开始出现。欧内斯特·博伊特勒博士在推动我们对铁调素功能的理解方面发挥了重要作用。本综述谨以此纪念他。

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本文引用的文献

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A ferroportin transcript that lacks an iron-responsive element enables duodenal and erythroid precursor cells to evade translational repression.一种缺乏铁反应元件的铁转运蛋白转录本使十二指肠和红系前体细胞能够逃避翻译抑制。
Cell Metab. 2009 May;9(5):461-73. doi: 10.1016/j.cmet.2009.03.006.
2
The molecular basis of hepcidin-resistant hereditary hemochromatosis.铁调素抵抗性遗传性血色素沉着症的分子基础。
Blood. 2009 Jul 9;114(2):437-43. doi: 10.1182/blood-2008-03-146134. Epub 2009 Apr 21.
3
Hepcidin, the hormone of iron metabolism, is bound specifically to alpha-2-macroglobulin in blood.铁调素是铁代谢的激素,在血液中特异性地与α-2-巨球蛋白结合。
Blood. 2009 Jun 11;113(24):6225-36. doi: 10.1182/blood-2009-01-201590. Epub 2009 Apr 20.
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BMP6 orchestrates iron metabolism.骨形态发生蛋白6调控铁代谢。
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5
Interaction of the hereditary hemochromatosis protein HFE with transferrin receptor 2 is required for transferrin-induced hepcidin expression.遗传性血色素沉着症蛋白HFE与转铁蛋白受体2的相互作用是转铁蛋白诱导的铁调素表达所必需的。
Cell Metab. 2009 Mar;9(3):217-27. doi: 10.1016/j.cmet.2009.01.010.
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Immunoassay for human serum hepcidin.人血清铁调素免疫测定法。
Blood. 2008 Nov 15;112(10):4292-7. doi: 10.1182/blood-2008-02-139915. Epub 2008 Aug 8.
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Dorsomorphin inhibits BMP signals required for embryogenesis and iron metabolism.多索茶碱抑制胚胎发育和铁代谢所需的骨形态发生蛋白信号。
Nat Chem Biol. 2008 Jan;4(1):33-41. doi: 10.1038/nchembio.2007.54. Epub 2007 Nov 18.
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Posttranslational processing of hepcidin in human hepatocytes is mediated by the prohormone convertase furin.人肝细胞中肝素的翻译后加工由激素原转化酶弗林蛋白酶介导。
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High levels of GDF15 in thalassemia suppress expression of the iron regulatory protein hepcidin.地中海贫血中高水平的生长分化因子15抑制铁调节蛋白铁调素的表达。
Nat Med. 2007 Sep;13(9):1096-101. doi: 10.1038/nm1629. Epub 2007 Aug 26.
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Regulation of iron homeostasis by the hypoxia-inducible transcription factors (HIFs).缺氧诱导转录因子(HIFs)对铁稳态的调节。
J Clin Invest. 2007 Jul;117(7):1926-32. doi: 10.1172/JCI31370.