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 article

112 Scopus citations

Abstract

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
Volume1797
Issue number6-7
DOIs
StatePublished - Jun 1 2010

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Keywords

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

ASJC Scopus subject areas

  • Biophysics
  • Biochemistry
  • Cell Biology

Cite this

Giorgio, V., Soriano, M. E., Basso, E., Bisetto, E., Lippe, G., Forte, M. A., & Bernardi, P. (2010). Cyclophilin D in mitochondrial pathophysiology. Biochimica et Biophysica Acta - Bioenergetics, 1797(6-7), 1113-1118. https://doi.org/10.1016/j.bbabio.2009.12.006