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Progress on the Mitochondrial Permeability Transition Pore: Regulation by Complex I and Ubiquinone Analogs

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Abstract

This review summarizes recent progress on the regulation of the mitochondrial permeabilitytransition pore, an inner membrane channel that may play a role in cell death. We brieflycover its key control points as emerged over the last few years from studies on isolatedmitochondria; and describe in some detail our recent results indicating that the pore is modulatedby the respiratory chain complex I and can be specifically blocked by selected ubiquinoneanalogs. We discuss the potential relevance of these findings for the structural definition ofthe permeability transition pore and illustrate the pharmacological perspectives they offer indiseases where mitochondrial dysfunction is suspected to play a key role.

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Authors and Affiliations

  1. Laboratoire de Bioénergétique Fondamentale et Appliquée, Université J. Fourier, F-38041, Grenoble, France

    Eric Fontaine

  2. Consiglio Nazionale delle Ricerche Unit for the Study of Biomembranes and the Laboratory of Biophysics and Membrane Biology, Department of Biomedical Sciences, University of Padova Medical School, I-35121, Padova, Italy

    Paolo Bernardi

  3. Dipartimento di Scienze Biomediche Sperimentali, Viale Giuseppe Colombo 3, I-35121, Padova, Italy

    Paolo Bernardi

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Fontaine, E., Bernardi, P. Progress on the Mitochondrial Permeability Transition Pore: Regulation by Complex I and Ubiquinone Analogs. J Bioenerg Biomembr 31, 335–345 (1999). https://doi.org/10.1023/A:1005475802350

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