The mitochondrial permeability transition from in vitro artifact to disease target.
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 approximately 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.
Bernardi
Paolo
P
Department of Biomedical Sciences and CNR Institute of Neurosciences, University of Padova, Italy. bernardi@bio.unipd.it
Krauskopf
Alexandra
A
Basso
Emy
E
Petronilli
Valeria
V
Blachly-Dyson
Elizabeth
E
Blalchy-Dyson
Elizabeth
E
Di Lisa
Fabio
F
Forte
Michael A
MA
D000818:Animals; D016477:Artifacts; D016503:Drug Delivery Systems; D006801:Humans; D008107:Liver Diseases / Q000188:drug therapy / Q000503:physiopathology; D033681:Mitochondrial Membrane Transport Proteins / Q000737:chemistry / Q000187:drug effects* / Q000502:physiology*; D000083162:Mitochondrial Permeability Transition Pore; D009135:Muscular Diseases / Q000188:drug therapy / Q000503:physiopathology; D017202:Myocardial Ischemia / Q000188:drug therapy / Q000503:physiopathology; D009422:Nervous System Diseases / Q000188:drug therapy / Q000503:physiopathology
D033681:Mitochondrial Membrane Transport Proteins; D000083162:Mitochondrial Permeability Transition Pore