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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Transferrin receptor is negatively modulated by the hemochromatosis protein HFE: Implications for cellular iron homeostasis
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Transferrin receptor is negatively modulated by the hemochromatosis protein HFE: Implications for cellular iron homeostasis

机译：转铁蛋白受体受血色素沉着病蛋白HFE负调节：对细胞铁稳态的影响

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摘要

Hereditary hemochromatosis is a common autosomal rccessive disorder of iron metabolism. Recent demonstration of an association between transferrin receptor (Tffe) and HFE, a major histocompatibility complex class I-Iike molecule that has been implicated to play a role in hereditary hemochro- matosis, further strengthens the notion that HFE is involved in iron metabolism. Herein we show that TfR is required for and controls the assembly and the intracellular transport and sun face expression of HFE. Because surface-expressed HFE and TfR remain firmly associated physically, only the fraction of TfR that is associated with HFE during biosynthesis is affected functionally. Moreover, we show that HFE binding reduces the number of functional transferrin binding sites and impairs TfR internal- ization, thus reducing the uptake of transferrin-bound iron. Thus, iron homeostasis is indirectly regulated by HFE, a negative modulator of TfR.
机译：遗传性血色素沉着病是铁代谢的常见常染色体继发性继发性疾病。最近证明了转铁蛋白受体（Tffe）和HFE之间的联系，HFE是一种主要的组织相容性复杂的I-Iike类分子，被认为在遗传性血色病中起作用，进一步证实了HFE与铁代谢有关。在本文中，我们显示TfR是HFE的装配和细胞内运输和太阳面表达所必需并对其进行控制。因为表面表达的HFE和TfR在物理上保持牢固的关联，所以在生物合成过程中仅与HFE关联的TfR部分在功能上受到影响。此外，我们表明HFE结合减少了功能性转铁蛋白结合位点的数量并损害了TfR的内在化，从而减少了与转铁蛋白结合的铁的吸收。因此，铁稳态由TFE的负调节剂HFE间接调节。

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