title: Respiratory chain inhibition: one more feature to propose MPTP intoxication as a Leigh syndrome model.
abstract: 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) intoxicated mice have been widely used to model the loss of dopaminergic neurons. As this treatment leads to basal ganglia degeneration, it was proposed that MPTP mice could be used as a model of Leigh syndrome. However, this mitochondrial pathology is biochemically characterized by a respiratory chain dysfunction. To determine if MPTP can affect in vivo mitochondria function, we measured the activities of mitochondrial respiratory chain complexes in several tissues. Our results show that MPTP affects mainly mitochondrial respiratory chain complex IV, as found in Leigh Syndrome, confirming that acute MPTP intoxicated mice are a good model of Leigh Syndrome.
authors: Métabolisme Energétique Cellulaire, Institut de Biochimie et Génétique Cellulaires, UMR 5095 CNRS/Univ. de Bordeaux, 1 Rue Camille Saint Saëns, CS 61390, F- 33000, Bordeaux, Cedex, France.|Equipe de Médecine Evolutive, AMIS, UMR 5288 CNRS/Univ. Paul Sabatier, F-31073, Toulouse, France.
authors: Barbara
authors: B
authors: Da Costa
authors: Métabolisme Energétique Cellulaire, Institut de Biochimie et Génétique Cellulaires, UMR 5095 CNRS/Univ. de Bordeaux, 1 Rue Camille Saint Saëns, CS 61390, F- 33000, Bordeaux, Cedex, France.|Equipe de Médecine Evolutive, AMIS, UMR 5288 CNRS/Univ. Paul Sabatier, F-31073, Toulouse, France.
authors: Elodie
authors: E
authors: Dumon
authors: UMR INSERM U 930, CNRS FRE 2448, François-Rabelais University, Child Neurology Unit, University Hospital, Tours, France.
authors: Laurence
authors: L
authors: Le Moigno
authors: UMR INSERM U 930, CNRS FRE 2448, François-Rabelais University, Child Neurology Unit, University Hospital, Tours, France.
authors: Sylvie
authors: S
authors: Bodard
authors: UMR INSERM U 930, CNRS FRE 2448, François-Rabelais University, Child Neurology Unit, University Hospital, Tours, France.
authors: Pierre
authors: P
authors: Castelnau
authors: Equipe de Médecine Evolutive, AMIS, UMR 5288 CNRS/Univ. Paul Sabatier, F-31073, Toulouse, France.
authors: Thierry
authors: T
authors: Letellier
authors: Métabolisme Energétique Cellulaire, Institut de Biochimie et Génétique Cellulaires, UMR 5095 CNRS/Univ. de Bordeaux, 1 Rue Camille Saint Saëns, CS 61390, F- 33000, Bordeaux, Cedex, France. christophe.rocher@u-bordeaux.fr.|Equipe de Médecine Evolutive, AMIS, UMR 5288 CNRS/Univ. Paul Sabatier, F-31073, Toulouse, France. christophe.rocher@u-bordeaux.fr.
authors: Christophe
authors: C
authors: Rocher
mesh_terms: D015632:1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine / Q000008:administration & dosage / Q000009:adverse effects*; D000818:Animals; D004195:Disease Models, Animal; D004579:Electron Transport / Q000187:drug effects*; D003576:Electron Transport Complex IV / Q000187:drug effects; D007888:Leigh Disease / Q000139:chemically induced*; D020267:MPTP Poisoning; D051379:Mice; D008928:Mitochondria / Q000378:metabolism
chemical_list: D015632:1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine; D003576:Electron Transport Complex IV
keywords: MPTP; Mice model; Mitochondrial diseases; Respiratory chain