Iron in Porphyrias: Friend or Foe?
Iron in Porphyrias: Friend or Foe?
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DOI：
10.3390/diagnostics12020272
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发表时间：
2022-01-21
期刊：
影响因子：
--
通讯作者：
Corradini E
中科院分区：
文献类型：
--
作者：
Buzzetti E;Ventura P;Corradini E
铁是一种微量元素，对许多重要过程都很重要，包括氧运输、氧化代谢、细胞增殖和催化反应。铁主要作为血红素分子的一部分支持这些功能。血红素合成是一个八步过程，当所涉及的八种酶之一的水平有缺陷时，会导致一组疾病的发展，无论是遗传性的还是获得性的，称为卟啉症。尽管铁和血红素之间存在严格的联系，但铁在不同类型卟啉症中的作用，特别是作为疾病发展/进展的风险因素或作为潜在的治疗靶点或分子，仍然存在争议，因为对比结果已经从临床观察，体外研究和动物模型中出现。在这篇综述中，我们的目的是加深这些方面提请注意铁在卟啉症的作用及其潜在的影响目前的证据。通过血液检查，成像技术或遗传研究对卟啉症患者进行铁状态及其代谢途径的检测可以为临床护理提供额外的诊断和预后价值，从而实现更有针对性和更有效的管理。
Iron is a trace element that is important for many vital processes, including oxygen transport, oxidative metabolism, cellular proliferation, and catalytic reactions. Iron supports these functions mainly as part of the heme molecule. Heme synthesis is an eight-step process which, when defective at the level of one of the eight enzymes involved, can cause the development of a group of diseases, either inherited or acquired, called porphyrias. Despite the strict link between iron and heme, the role of iron in the different types of porphyrias, particularly as a risk factor for disease development/progression or as a potential therapeutic target or molecule, is still being debated, since contrasting results have emerged from clinical observations, in vitro studies and animal models. In this review we aim to deepen such aspects by drawing attention to the current evidence on the role of iron in porphyrias and its potential implication. Testing for iron status and its metabolic pathways through blood tests, imaging techniques or genetic studies on patients affected by porphyrias can provide additional diagnostic and prognostic value to the clinical care, leading to a more tailored and effective management.
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