Mitochondrial Iron in Human Health and Disease.
Mitochondrial Iron in Human Health and Disease.
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DOI：
10.1146/annurev-physiol-020518-114742
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发表时间：
2019-02
影响因子：
18.2
通讯作者：
D. Ward;S. Cloonan
中科院分区：
文献类型：
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作者：
D. Ward;S. Cloonan
线粒体是真核细胞的标志性区别特征。线粒体包含一个活跃的细胞器网络，其融合、分裂和指导无数重要的生物学功能，包括能量代谢、细胞死亡调节和不同组织中的先天免疫信号传导。这些动态细胞器的另一个重要但经常被低估的功能是它们在哺乳动物细胞中最丰富和生物学上最通用的过渡金属铁代谢中的核心作用。近年来，线粒体铁功能障碍的细胞和动物模型为鉴定新蛋白质提供了重要信息，这些蛋白质阐明了线粒体稳态和铁代谢所涉及的途径。与疾病发病机制和/或进展相关的线粒体铁失调的特定特征变得越来越重要。了解调节线粒体铁途径的分子机制将有助于更好地确定这种重要金属在线粒体功能和人类健康和疾病中的作用。
Mitochondria are an iconic distinguishing feature of eukaryotic cells. Mitochondria encompass an active organellar network that fuses, divides, and directs a myriad of vital biological functions, including energy metabolism, cell death regulation, and innate immune signaling in different tissues. Another crucial and often underappreciated function of these dynamic organelles is their central role in the metabolism of the most abundant and biologically versatile transition metals in mammalian cells, iron. In recent years, cellular and animal models of mitochondrial iron dysfunction have provided vital information in identifying new proteins that have elucidated the pathways involved in mitochondrial homeostasis and iron metabolism. Specific signatures of mitochondrial iron dysregulation that are associated with disease pathogenesis and/or progression are becoming increasingly important. Understanding the molecular mechanisms regulating mitochondrial iron pathways will help better define the role of this important metal in mitochondrial function and in human health and disease.