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线粒体通透性转换:从体外人为现象到疾病靶点

The mitochondrial permeability transition from in vitro artifact to disease target.

作者信息

Bernardi Paolo, Krauskopf Alexandra, Basso Emy, Petronilli Valeria, Blachly-Dyson Elizabeth, Di Lisa Fabio, Forte Michael A

机构信息

Department of Biomedical Sciences and CNR Institute of Neurosciences, University of Padova, Italy.

出版信息

FEBS J. 2006 May;273(10):2077-99. doi: 10.1111/j.1742-4658.2006.05213.x.

DOI:10.1111/j.1742-4658.2006.05213.x
PMID:16649987
Abstract

The mitochondrial permeability transition pore is a high conductance channel whose opening leads to an increase of mitochondrial inner membrane permeability to solutes with molecular masses up to approximately 1500 Da. In this review we trace the rise of the permeability transition pore from the status of in vitro artifact to that of effector mechanism of cell death. We then cover recent results based on genetic inactivation of putative permeability transition pore components, and discuss their meaning for our understanding of pore structure. Finally, we discuss evidence indicating that the permeability transition pore plays a role in pathophysiology, with specific emphasis on in vivo models of disease.

摘要

线粒体通透性转换孔是一种高电导通道,其开放会导致线粒体内膜对分子量高达约1500道尔顿的溶质的通透性增加。在本综述中,我们追溯了通透性转换孔从体外人工产物状态到细胞死亡效应机制状态的兴起过程。然后,我们介绍了基于假定的通透性转换孔成分基因失活的最新研究结果,并讨论了它们对于我们理解孔结构的意义。最后,我们讨论了表明通透性转换孔在病理生理学中起作用的证据,特别强调了疾病的体内模型。

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