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Updated: Aug 15, 2026

Assessment of Open Probability of the Mitochondrial Permeability Transition Pore in the Setting of Coenzyme Q Excess
Published on: June 1, 2022
Mitochondrial ion channels: gatekeepers of life and death
Brian O'Rourke1, Sonia Cortassa, Miguel A Aon
1Institute of Molecular Cardiobiology, Division of Cardiology, The Johns Hopkins University, Baltimore, Maryland, USA. bor@jhmi.edu
Abstract:
Continuous generation of ATP by mitochondrial oxidative phosphorylation is essential to maintain function in mechanically active cells such as cardiomyocytes. Emerging evidence indicates that mitochondrial ion channels activated by reactive oxygen species can induce a mitochondrial "critical" state, which can scale to cause electrical and contractile dysfunction of the cardiac cell and, ultimately, the whole heart. Here we focus on how mitochondrial ion channels participate in life-and-death decisions of the cell and discuss the challenges ahead for translating recent findings into novel therapeutic applications.
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