title: Physiological and pathological roles of the mitochondrial permeability transition pore in the heart.
abstract: Prolonged mitochondrial permeability transition pore (MPTP) opening results in mitochondrial energetic dysfunction, organelle swelling, rupture, and typically a type of necrotic cell death. However, acute opening of the MPTP has a critical physiologic role in regulating mitochondrial Ca(2+) handling and metabolism. Despite the physiological and pathological roles that the MPTP orchestrates, the proteins that comprise the pore itself remain an area of ongoing investigation. Here, we will discuss the molecular composition of the MPTP and its role in regulating cardiac physiology and disease. A better understanding of MPTP structure and function will likely suggest novel cardioprotective therapeutic approaches.
authors: Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH 45229, USA.
authors: Jennifer Q
authors: JQ
authors: Kwong
authors: Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH 45229, USA; Howard Hughes Medical Institute, Cincinnati, OH 45229, USA. Electronic address: jeff.molkentin@cchmc.org.
authors: Jeffery D
authors: JD
authors: Molkentin
mesh_terms: D000818:Animals; D002118:Calcium / Q000378:metabolism; D006321:Heart / Q000502:physiology*; D006801:Humans; D008929:Mitochondria, Heart / Q000378:metabolism*; D033681:Mitochondrial Membrane Transport Proteins / Q000378:metabolism*; D000083162:Mitochondrial Permeability Transition Pore; D009206:Myocardium / Q000378:metabolism* / Q000473:pathology*
chemical_list: D033681:Mitochondrial Membrane Transport Proteins; D000083162:Mitochondrial Permeability Transition Pore; D002118:Calcium
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