Titre :
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Mobilization of adenine nucleotide translocators as one molecular mechanism of the biochemical threshold effect observed in mitochondrial diseases (abstract : congrès international de Myologie, 2005)
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contenu dans :
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Auteurs :
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Congrès international de myologie 2005 (International Congress of Myology 2005; 9-13 mai 2005; Nantes, France) ;
Letellier T ;
Faustin B ;
Rocher C ;
Bénard G ;
Malgat M ;
Rossignol R
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Type de document :
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Article
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Année de publication :
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2005
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Pages :
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p. 172
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Langues:
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Anglais
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Mots-clés :
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adénine nucléotide translocase
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biologie moléculaire
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chaîne respiratoire
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colloque
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mécanisme d'action
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métabolisme énergétique
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mitochondrie
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myopathie mitochondriale
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Résumé :
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Communication n° 7. The existence of a biochemical threshold effect in the metabolic expression of oxidative phosphorylation deficiencies has considerable implications for the understanding of mitochondrial bioenergetics and the study of mitochondrial diseases. However, the molecular bases of this phenomenon remain unclear. We report here a new mechanism to explain this threshold effect, based on a reserve of enzymes not initially participating in the respiratory rate that can be activated either to respond to a flux increase or to compensate for a defect induced by a mutation. We show that this mobilization occurs through: 1) the assembly of inactive adenine nucleotide translocator isoform 1 subunits into oligomeric active carriers 2) conformational changes in the adenine nucleotide translocator isoform 1 in a permeability transition pore-like structure. We discuss how these transitions are sensitive to the steady state of oxidative phosphorylation functioning or tissue and analyze their consequences on the threshold effect.
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