The mitochondrial permeability transition pore (mPTP) or mitochondrial megachannel is arguably one of the most mysterious phenomena in biology today. mPTP has been at the center of ongoing extensive scientific research for the last several decades. In this review we will discuss recent advances in the field that enhance our understanding of the molecular composition of mPTP, its regulatory mechanisms and its pathophysiological role. We will describe our recent findings on the role of ATP synthase c-subunit ring as a central player in mitochondrial permeability transition and as an important metabolic regulator during development and in degenerative diseases.

线粒体通透性转换孔 (mPTP) 或线粒体巨通道可以说是当今生物学中最神秘的现象之一。在过去的几十年里，mPTP 一直处于正在进行的广泛科学研究的中心。在这篇综述中，我们将讨论该领域的最新进展，这些进展增强了我们对 mPTP 的分子组成、其调节机制及其病理生理学作用的理解。我们将描述我们最近关于 ATP 合酶 c-亚基环作为线粒体通透性转变中的核心参与者以及在发育和退行性疾病中作为重要代谢调节剂的作用的发现。