Cell death disguised: The mitochondrial permeability transition pore as the c-subunit of the F(1)F(O) ATP synthase.
Cell death disguised: The mitochondrial permeability transition pore as the c-subunit of the F(1)F(O) ATP synthase.
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DOI：
10.1016/j.phrs.2015.04.013
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发表时间：
2015-09
影响因子：
9.3
通讯作者：
Alavian KN
中科院分区：
文献类型：
--
作者：
Jonas EA;Porter GA Jr;Beutner G;Mnatsakanyan N;Alavian KN
跨线粒体内外膜的离子运输是线粒体功能的核心，包括氧化磷酸化和细胞死亡的调节。虽然线粒体产生ATP是必需的，但最近的研究证实，ATP合成酶的c-亚基也包含一个大电导解偶联通道，即线粒体通透性转换孔(MPTP)，其持续的开放会导致线粒体内膜的渗透调节失调和细胞死亡。本文将对MPTP的分子组成、调控机制以及如何直接参与其生理和病理作用等方面的最新进展进行综述。
Ion transport across the mitochondrial inner and outer membranes is central to mitochondrial function, including regulation of oxidative phosphorylation and cell death. Although essential for ATP production by mitochondria, recent findings have confirmed that the c-subunit of the ATP synthase also houses a large conductance uncoupling channel, the mitochondrial permeability transition pore (mPTP), the persistent opening of which produces osmotic dysregulation of the inner mitochondrial membrane and cell death. This review will discuss recent advances in understanding the molecular components of mPTP, its regulatory mechanisms and how these contribute directly to its physiological as well as pathological roles.