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

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.