Respiratory chain inhibition: one more feature to propose MPTP intoxication as a Leigh syndrome model |
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Authors: | Barbara Da Costa Elodie Dumon Laurence Le Moigno Sylvie Bodard Pierre Castelnau Thierry Letellier Christophe Rocher |
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Institution: | 1.Métabolisme Energétique Cellulaire, Institut de Biochimie et Génétique Cellulaires,UMR 5095 CNRS/Univ. de Bordeaux,Bordeaux,France;2.Equipe de Médecine Evolutive,AMIS, UMR 5288 CNRS/Univ. Paul Sabatier,Toulouse,France;3.UMR INSERM U 930, CNRS FRE 2448,Fran?ois-Rabelais University, Child Neurology Unit, University Hospital,Tours,France |
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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. |
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