Cyclophilin D in mitochondrial pathophysiology

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• 作者：Valentina Giorgio ; Maria Eugenia Soriano ; Emy Basso ; Elena Bisetto ; Giovanna Lippe ; Michael A. Forte ; Paolo Bernardi
• 关键词：Cyclophilin ; Mitochondria ; ATP synthase ; Permeability transition ; Cyclosporin A
• 刊名：Biochimica et Biophysica Acta (BBA)/Bioenergetics
• 出版年：2010
• 出版时间：June-July 2010
• 年：2010
• 卷：1797
• 期：6-7
• 页码：1113-1118
• 全文大小：336 K

文摘

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.