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lüll Cyclophilin D in mitochondrial pathophysiology Giorgio V; Soriano ME; Basso E; Bisetto E; Lippe G; Forte MA; Bernardi PBiochim Biophys Acta 2010[Jun]; 1797 (6-7): 1113-8Cyclophilins 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.|Animals[MESH]|Calcineurin/metabolism[MESH]|Cyclophilins/deficiency/genetics/*metabolism[MESH]|Cyclosporine/metabolism[MESH]|Disease Models, Animal[MESH]|Humans[MESH]|Mice[MESH]|Mice, Knockout[MESH]|Mitochondria/*metabolism[MESH]|Mitochondrial Diseases/genetics/metabolism[MESH]|Mitochondrial Membrane Transport Proteins/metabolism[MESH]|Mitochondrial Permeability Transition Pore[MESH]|Mitochondrial Proton-Translocating ATPases/metabolism[MESH]|Models, Biological[MESH]|Peptidyl-Prolyl Isomerase F[MESH]|Protein Interaction Domains and Motifs[MESH] |