Cyclophilin D in mitochondrial pathophysiology

Valentina Giorgio, Maria Eugenia Soriano, Emy Basso, Elena Bisetto, Giovanna Lippe, Michael A. Forte, Paolo Bernardi

Research output: Contribution to journalReview articlepeer-review

147 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1113-1118
Number of pages6
JournalBiochimica et Biophysica Acta - Bioenergetics
Issue number6-7
StatePublished - Jun 2010
Externally publishedYes


  • ATP synthase
  • Cyclophilin
  • Cyclosporin A
  • Mitochondria
  • Permeability transition

ASJC Scopus subject areas

  • Biophysics
  • Biochemistry
  • Cell Biology


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