Mitochondrial ferritin
Mitochondrial ferritin
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DOI：
10.1016/j.biocel.2003.10.020
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发表时间：
2004-10-01
影响因子：
4
通讯作者：
Arosio, P
中科院分区：
文献类型：
--
作者：
Levi, S;Arosio, P
最近在人和小鼠体内发现了一种专门针对线粒体的新型铁蛋白。它在结构和功能上与胞质铁蛋白相似，胞质铁蛋白是大多数生命系统中发现的特征良好的分子，旨在储存和解毒细胞铁。哺乳动物中胞浆铁蛋白无处不在，而线粒体铁蛋白表达主要限于睾丸、神经元细胞和胰岛。此外，铁粒幼细胞贫血患者的成红细胞的铁负载线粒体中也含有丰富的铁。重组和转染的线粒体铁蛋白的表征表明，该蛋白具有保护线粒体免受感染的作用。铁引起的损伤。这些数据表明，它是研究该细胞器中铁代谢的一个有趣的工具。此外，它可能有助于诊断骨髓增生异常综合征和保护线粒体免受过量铁的毒性作用。 (C) 2003 Elsevier Ltd. 保留所有权利。
A novel ferritin type specifically targeted to mitochondria has been recently found in human and mouse. It is structurally and functionally similar to the cytosolic ferritins, well-characterized molecules found in most living systems which are designed to store and detoxify cellular iron. Cytosolic ferritins in mammals are ubiquitous while mitochondrial ferritin expression is restricted mainly to the testis, neuronal cells and islets of Langherans. In addition, it is abundant in the iron-loaded mitochondria of erythroblasts of patients with sideroblastic anaemia. The characterization of recombinant and transfected mitochondrial ferritin indicated that this protein has a role in protecting mitochondria from. iron-induced damage. These data suggest that it is an interesting tool to study the iron metabolism in this organelle. In addition, it may be useful for the diagnosis of myelodysplastic syndromes and in protecting mitochondria from the toxic effects of excess iron. (C) 2003 Elsevier Ltd. All rights reserved.