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Hfe-KO 小鼠中的红细胞生成增强表明 Hfe 在调节红细胞铁稳态中的作用。

Enhanced erythropoiesis in Hfe-KO mice indicates a role for Hfe in the modulation of erythroid iron homeostasis.

机构信息

Department of Pediatrics Hematology-Oncology, Children's Blood Foundation Laboratories, Weill Medical College of Cornell University, New York, NY, USA.

出版信息

Blood. 2011 Jan 27;117(4):1379-89. doi: 10.1182/blood-2010-09-307462. Epub 2010 Nov 8.


DOI:10.1182/blood-2010-09-307462
PMID:21059897
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3056475/
Abstract

In hereditary hemochromatosis, mutations in HFE lead to iron overload through abnormally low levels of hepcidin. In addition, HFE potentially modulates cellular iron uptake by interacting with transferrin receptor, a crucial protein during erythropoiesis. However, the role of HFE in this process was never explored. We hypothesize that HFE modulates erythropoiesis by affecting dietary iron absorption and erythroid iron intake. To investigate this, we used Hfe-KO mice in conditions of altered dietary iron and erythropoiesis. We show that Hfe-KO mice can overcome phlebotomy-induced anemia more rapidly than wild-type mice (even when iron loaded). Second, we evaluated mice combining the hemochromatosis and β-thalassemia phenotypes. Our results suggest that lack of Hfe is advantageous in conditions of increased erythropoietic activity because of augmented iron mobilization driven by deficient hepcidin response. Lastly, we demonstrate that Hfe is expressed in erythroid cells and impairs iron uptake, whereas its absence exclusively from the hematopoietic compartment is sufficient to accelerate recovery from phlebotomy. In summary, we demonstrate that Hfe influences erythropoiesis by 2 distinct mechanisms: limiting hepcidin expression under conditions of simultaneous iron overload and stress erythropoiesis, and impairing transferrin-bound iron uptake by erythroid cells. Moreover, our results provide novel suggestions to improve the treatment of hemochromatosis.

摘要

在遗传性血色素沉着症中,HFE 的突变导致铁过载,原因是铁调素水平异常降低。此外,HFE 可能通过与转铁蛋白受体相互作用来调节细胞铁摄取,转铁蛋白受体是红细胞生成过程中的关键蛋白。然而,HFE 在这个过程中的作用从未被探索过。我们假设 HFE 通过影响膳食铁吸收和红系铁摄入来调节红细胞生成。为了研究这一点,我们在改变膳食铁和红细胞生成的条件下使用 Hfe-KO 小鼠。我们表明,Hfe-KO 小鼠可以比野生型小鼠更快地克服放血引起的贫血(即使铁负荷)。其次,我们评估了结合血色素沉着症和β-地中海贫血表型的小鼠。我们的结果表明,由于缺乏铁调素反应导致铁动员增加,因此在红细胞生成活性增加的情况下缺乏 Hfe 是有利的。最后,我们证明 Hfe 在红细胞中表达并损害铁摄取,而其仅从造血细胞中缺失足以加速从放血中恢复。总之,我们证明 Hfe 通过 2 种不同的机制影响红细胞生成:在同时铁过载和应激性红细胞生成的情况下限制铁调素表达,以及损害红细胞的转铁蛋白结合铁摄取。此外,我们的结果为改善血色素沉着症的治疗提供了新的建议。

相似文献

[1]
Enhanced erythropoiesis in Hfe-KO mice indicates a role for Hfe in the modulation of erythroid iron homeostasis.

Blood. 2010-11-8

[2]
The transferrin receptor modulates Hfe-dependent regulation of hepcidin expression.

Cell Metab. 2008-3

[3]
An RNAi therapeutic targeting Tmprss6 decreases iron overload in Hfe(-/-) mice and ameliorates anemia and iron overload in murine β-thalassemia intermedia.

Blood. 2012-12-6

[4]
Interaction of the hereditary hemochromatosis protein HFE with transferrin receptor 2 is required for transferrin-induced hepcidin expression.

Cell Metab. 2009-3

[5]
Function of the hemochromatosis protein HFE: Lessons from animal models.

World J Gastroenterol. 2008-12-7

[6]
Distinct requirements for Hfe in basal and induced hepcidin levels in iron overload and inflammation.

Am J Physiol Gastrointest Liver Physiol. 2006-8

[7]
Combined deletion of Hfe and transferrin receptor 2 in mice leads to marked dysregulation of hepcidin and iron overload.

Hepatology. 2009-12

[8]
Physiologic systemic iron metabolism in mice deficient for duodenal Hfe.

Blood. 2007-5-15

[9]
Hepcidin and Hfe in iron overload in beta-thalassemia.

Ann N Y Acad Sci. 2010-8

[10]
Disruption of hemochromatosis protein and transferrin receptor 2 causes iron-induced liver injury in mice.

Hepatology. 2012-6-11

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IUBMB Life. 2025-1

[2]
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Nat Commun. 2024-5-10

[3]
Iron overload induces dysplastic erythropoiesis and features of myelodysplasia in Nrf2-deficient mice.

Leukemia. 2024-1

[4]
Ferritin Increase in Hemochromatosis Subjects After Discontinuing Their Regular Maintenance Treatment: A Longitudinal Analysis Performed During the COVID-19 Imposed Hospital Lockdown.

Hemasphere. 2022-8-23

[5]
Cell-specific expression of determines the outcome of serovar Typhimurium infection in mice.

Haematologica. 2021-12-1

[6]
What can we learn from ineffective erythropoiesis in thalassemia?

Blood Rev. 2017-10-3

[7]
Blood Transcriptomic Meta-analysis Identifies Dysregulation of Hemoglobin and Iron Metabolism in Parkinson' Disease.

Front Aging Neurosci. 2017-3-29

[8]
Genetic control of erythropoiesis.

Curr Opin Hematol. 2017-5

[9]
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Cell. 2017-1-26

[10]
HFE gene: Structure, function, mutations, and associated iron abnormalities.

Gene. 2015-12-15

本文引用的文献

[1]
Transferrin receptor 2 is a component of the erythropoietin receptor complex and is required for efficient erythropoiesis.

Blood. 2010-9-8

[2]
Combined deletion of Hfe and transferrin receptor 2 in mice leads to marked dysregulation of hepcidin and iron overload.

Hepatology. 2009-12

[3]
Resolving the distinct stages in erythroid differentiation based on dynamic changes in membrane protein expression during erythropoiesis.

Proc Natl Acad Sci U S A. 2009-10-13

[4]
A ferroportin transcript that lacks an iron-responsive element enables duodenal and erythroid precursor cells to evade translational repression.

Cell Metab. 2009-5

[5]
Interaction of the hereditary hemochromatosis protein HFE with transferrin receptor 2 is required for transferrin-induced hepcidin expression.

Cell Metab. 2009-3

[6]
Contribution of STAT3 and SMAD4 pathways to the regulation of hepcidin by opposing stimuli.

Blood. 2009-4-9

[7]
Exogenous iron increases hemoglobin in beta-thalassemic mice.

Exp Hematol. 2009-2

[8]
Stat5 regulates cellular iron uptake of erythroid cells via IRP-2 and TfR-1.

Blood. 2008-11-1

[9]
Erythroblastic islands: niches for erythropoiesis.

Blood. 2008-8-1

[10]
Iron regulates phosphorylation of Smad1/5/8 and gene expression of Bmp6, Smad7, Id1, and Atoh8 in the mouse liver.

Blood. 2008-8-15

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