Mitochondrial inner membrane protein, Mic60/mitofilin in mammalian organ protection.

Mitochondrial inner membrane protein, Mic60/mitofilin in mammalian organ protection.
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DOI:
10.1002/jcp.27314
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发表时间:
2019-04
影响因子:
5.6
通讯作者:
Bopassa JC
Bopassa JC
中科院分区:
生物学2区
文献类型:
--
作者:
Feng Y;Madungwe NB;Bopassa JC

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线粒体内膜中线粒体接触位点和嵴组织系统(MICOS)的鉴定揭示了线粒体形成其签名嵴所需的复杂组分,其中许多蛋白质复合物包括电子传递链都位于嵴中。Mic 60/mitofilin被描述为MICOS组装和维持的核心组分,从而控制嵴形态、蛋白质转运、线粒体DNA转录以及连接线粒体内外膜。虽然Mic 60同源物存在于许多物种中,但哺乳动物Mic 60只是最近才作为几种器官系统和器官缺陷起源疾病的关键参与者而受到关注。在这篇综述中,我们总结了目前已知的关于Mic 60在哺乳动物中不断扩大的作用,并强调了一些新的研究,推动线粒体嵴组织领域朝着潜在的新的和令人兴奋的疗法靶向这种蛋白质。
The identification of the mitochondrial contact site and cristae organizing system (MICOS) in the inner mitochondrial membrane shed light on the intricate components necessary for mitochondria to form their signature cristae in which many protein complexes including the electron transport chain are localized. Mic60/mitofilin has been described as the core component for the assembly and maintenance of MICOS, thus controlling cristae morphology, protein transport, mitochondrial DNA transcription, as well as connecting the inner and outer mitochondrial membranes. Although Mic60 homologues are present in many species, mammalian Mic60 is only recently gaining attention as a critical player in several organ systems and diseases with mitochondrial-defect origins. In this review, we summarize what is currently known about the ever-expanding role of Mic60 in mammals, and highlight some new studies pushing the field of mitochondrial cristae organization towards potentially new and exciting therapies targeting this protein.
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