The mitochondrial permeability transition from in vitro artifact to disease target

Paolo Bernardi, Alexandra Krauskopf, Emy Basso, Valeria Petronilli, Elizabeth Blalchy-Dyson, Fabio Di Lisa, Michael A. Forte

Research output: Contribution to journalReview articlepeer-review

504 Scopus citations

Abstract

The mitochondrial permeability transition pore is a high conductance channel whose opening leads to an increase of mitochondrial inner membrane permeability to solutes with molecular masses up to ≈1500 Da. In this review we trace the rise of the permeability transition pore from the status of in vitro artifact to that of effector mechanism of cell death. We then cover recent results based on genetic inactivation of putative permeability transition pore components, and discuss their meaning for our understanding of pore structure. Finally, we discuss evidence indicating that the permeability transition pore plays a role in pathophysiology, with specific emphasis on in vivo models of disease.

Original languageEnglish (US)
Pages (from-to)2077-2099
Number of pages23
JournalFEBS Journal
Volume273
Issue number10
DOIs
StatePublished - May 2006

Keywords

  • Apoptosis
  • Calcium
  • Cancer
  • Cell death
  • Degenerative diseases
  • Drugs
  • Mitochondria
  • Necrosis
  • Permeability transition

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Cell Biology

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