Mitochondrial-associated metabolic disorders: foundations, pathologies and recent progress.

Mitochondrial-associated metabolic disorders: foundations, pathologies and recent progress.
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DOI:
10.1186/1743-7075-10-63
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发表时间:
2013-10-12
影响因子:
4.5
通讯作者:
McInnes J
McInnes J
中科院分区:
医学3区
文献类型:
--
作者:
McInnes J

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过去十年的研究彻底改变了我们看待线粒体的方式。线粒体不再仅仅被看作是细胞的发电站;相反,线粒体现在被认为是充满活力的、移动的结构,不断经历融合和裂变,并与其他细胞区室和结构进行密切的相互作用。研究结果表明,线粒体参与了多种细胞过程和分子相互作用,如钙缓冲、脂质流动和细胞内信号传导。因此,越来越多的人类病理学与线粒体的功能缺陷相关并不令人惊讶。理解和治疗由线粒体功能障碍引起的人类病理的困难来自线粒体和其他细胞过程之间的复杂关系,以及此类疾病的遗传背景。本文综述了线粒体功能缺陷或不足引起的代谢性疾病相关的线粒体过程的背景知识和最新进展,以及对当前和未来研究途径的见解。
Research in the last decade has revolutionized the way in which we view mitochondria. Mitochondria are no longer viewed solely as cellular powerhouses; rather, mitochondria are now understood to be vibrant, mobile structures, constantly undergoing fusion and fission, and engaging in intimate interactions with other cellular compartments and structures. Findings have implicated mitochondria in a wide variety of cellular processes and molecular interactions, such as calcium buffering, lipid flux, and intracellular signaling. As such, it does not come as a surprise that an increasing number of human pathologies have been associated with functional defects in mitochondria. The difficulty in understanding and treating human pathologies caused by mitochondrial dysfunction arises from the complex relationships between mitochondria and other cellular processes, as well as the genetic background of such diseases. This review attempts to provide a summary of the background knowledge and recent developments in mitochondrial processes relating to mitochondrial-associated metabolic diseases arising from defects or deficiencies in mitochondrial function, as well as insights into current and future avenues for investigation.
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