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 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.
Publication types
-
Research Support, N.I.H., Extramural
-
Research Support, Non-U.S. Gov't
-
Review
MeSH terms
-
Animals
-
Artifacts
-
Drug Delivery Systems*
-
Humans
-
Liver Diseases / drug therapy
-
Liver Diseases / physiopathology
-
Mitochondrial Membrane Transport Proteins / chemistry
-
Mitochondrial Membrane Transport Proteins / drug effects*
-
Mitochondrial Membrane Transport Proteins / physiology*
-
Mitochondrial Permeability Transition Pore
-
Muscular Diseases / drug therapy
-
Muscular Diseases / physiopathology
-
Myocardial Ischemia / drug therapy
-
Myocardial Ischemia / physiopathology
-
Nervous System Diseases / drug therapy
-
Nervous System Diseases / physiopathology
Substances
-
Mitochondrial Membrane Transport Proteins
-
Mitochondrial Permeability Transition Pore