Key Laboratory of Laboratory Medicine, Ministry of Education of China, Zhejiang Provincial Key Laboratory of Medical Genetics, School of Laboratory Medicine and Life Science, Wenzhou Medical College, Wenzhou, Zhejiang, China.
PLoS One. 2012;7(1):e29666. doi: 10.1371/journal.pone.0029666. Epub 2012 Jan 11.
Iron is essential for organisms. It is mainly utilized in mitochondria for biosynthesis of iron-sulfur clusters, hemes and other cofactors. Mitoferrin 1 and mitoferrin 2, two homologues proteins belonging to the mitochondrial solute carrier family, are required for iron delivery into mitochondria. Mitoferrin 1 is highly expressed in developing erythrocytes which consume a large amount of iron during hemoglobinization. Mitoferrin 2 is ubiquitously expressed, whose functions are less known. Zebrafish with mitoferrin 1 mutation show profound hypochromic anaemia and erythroid maturation arrests, and yeast with defects in MRS3/4, the counterparts of mitoferrin 1/2, has low mitochondrial iron levels and grows poorly by iron depletion. Mitoferrin 1 expression is up-regulated in yeast and mouse models of Fiedreich's ataxia disease and in human cell culture models of Parkinson disease, suggesting its involvement in the pathogenesis of diseases with mitochondrial iron accumulation. In this study we found that reduced mitoferrin levels in C. elegans by RNAi treatment causes pleiotropic phenotypes such as small body size, reduced fecundity, slow movement and increased sensitivity to paraquat. Despite these abnormities, lifespan was increased by 50% to 80% in N2 wild type strain, and in further studies using the RNAi sensitive strain eri-1, more than doubled lifespan was observed. The pathways or mechanisms responsible for the lifespan extension and other phenotypes of mitoferrin RNAi worms are worth further study, which may contribute to our understanding of aging mechanisms and the pathogenesis of iron disorder related diseases.
铁对于生物体来说是必不可少的。它主要在线粒体中用于铁硫簇、血红素和其他辅因子的生物合成。两种同源蛋白——线粒体铁蛋白 1 和线粒体铁蛋白 2,属于线粒体溶质载体家族,它们对于铁向线粒体的输送是必需的。线粒体铁蛋白 1 在发育中的红细胞中高度表达,这些红细胞在血红蛋白化过程中消耗大量的铁。线粒体铁蛋白 2 广泛表达,但其功能知之甚少。线粒体铁蛋白 1 突变的斑马鱼表现出严重的低色素性贫血和红细胞成熟停滞,而酵母中 MRS3/4(线粒体铁蛋白 1/2 的对应物)的缺陷导致线粒体铁水平降低,并且在缺铁时生长不良。在弗里德赖希共济失调症的酵母和小鼠模型以及帕金森病的人类细胞培养模型中,线粒体铁蛋白 1 的表达上调,表明其参与了线粒体铁积累相关疾病的发病机制。在这项研究中,我们发现通过 RNAi 处理降低秀丽隐杆线虫中的线粒体铁蛋白水平会导致多种表型,如体型小、繁殖力降低、运动缓慢和对百草枯的敏感性增加。尽管存在这些异常,但 N2 野生型菌株的寿命延长了 50%至 80%,而在进一步使用 RNAi 敏感菌株 eri-1 的研究中,寿命延长了一倍以上。值得进一步研究负责线粒体铁蛋白 RNAi 线虫寿命延长和其他表型的途径或机制,这可能有助于我们理解衰老机制和与铁紊乱相关疾病的发病机制。