title: The mitochondrial ADP/ATP carrier: functional and structural studies in the route of elucidating pathophysiological aspects.
abstract: The mitochondrial ADP/ATP carrier plays a central role in aerobic cell energetics by providing to the cytosol the ATP generated by oxidative phosphorylation. Though discovered around 40 years ago owing to the existence of unique inhibitors and in spite of numerous experimental approaches, this carrier, which stands as a model of the mitochondrial solute carriers keeps some long-standing mystery. There are still open challenging questions among them the precise ADP/ATP transport mechanism, the functional oligomeric state of the carrier and relationships between human ADP/ATP carrier dysfunctioning and pathologies. Deciphering the 3D structure of this carrier afforded a considerable progress of the knowledge but requires now additional data focused on molecular dynamics from this static picture. State of the art in this topic is reviewed and debated in this paper in view of better comprehending origin of the discrepancies in these questions and, finally, the multiple physiological roles of this carrier in eukaryotic cell economy.
authors.affiliation: Laboratoire de Physiologie Moléculaire et Cellulaire, Institut de Biochimie et Génétique Cellulaires-UMR 5095, CNRS-Université Bordeaux2, Bordeaux Cedex, France. vero.trezeguet@ibgc.u-bordeaux2.fr
authors.forename: Véronique
authors.initials: V
authors.lastname: Trézéguet
authors.forename: Ludovic
authors.initials: L
authors.lastname: Pélosi
authors.forename: Guy J M
authors.initials: GJ
authors.lastname: Lauquin
authors.forename: Gérard
authors.initials: G
authors.lastname: Brandolin
mesh_terms: D000818:Animals; D017124:Conserved Sequence; D019143:Evolution, Molecular; D006801:Humans; D008928:Mitochondria / Q000201:enzymology / Q000235:genetics; D000226:Mitochondrial ADP, ATP Translocases / Q000737:chemistry* / Q000172:deficiency / Q000235:genetics / Q000502:physiology*; D028361:Mitochondrial Diseases / Q000201:enzymology / Q000235:genetics; D008958:Models, Molecular; D009154:Mutation; D010085:Oxidative Phosphorylation; D020836:Protein Structure, Quaternary
chemical_list: D000226:Mitochondrial ADP, ATP Translocases