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Biochim Biophys Acta. 2010 Jun-Jul;1797(6-7):1113-8. doi: 10.1016/j.bbabio.2009.12.006. Epub 2009 Dec 21.

Cyclophilin D in mitochondrial pathophysiology.

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


Cyclophilins are a family of peptidyl-prolyl cis-trans isomerases whose enzymatic activity can be inhibited by cyclosporin A. Sixteen cyclophilins have been identified in humans, and cyclophilin D is a unique isoform that is imported into the mitochondrial matrix. Here we shall (i) review the best characterized functions of cyclophilin D in mitochondria, i.e. regulation of the permeability transition pore, an inner membrane channel that plays an important role in the execution of cell death; (ii) highlight new regulatory interactions that are emerging in the literature, including the modulation of the mitochondrial F1FO ATP synthase through an interaction with the lateral stalk of the enzyme complex; and (iii) discuss diseases where cyclophilin D plays a pathogenetic role that makes it a suitable target for pharmacologic intervention.

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