title: Free radicals-mediated damage in transmitochondrial cells harboring the T14487C mutation in the ND6 gene of mtDNA.
abstract: We have studied the production of reactive oxygen species (ROS) in transmitochondrial cells, harboring homoplasmic levels of the T14487C mtDNA mutation in the ND6 gene of mitochondrial DNA (mtDNA). Previous work has shown that this mutation causes complex I deficiency. Here, we show that this mutation causes an overproduction of ROS leading to an increase in the oxidation of lipids and mtDNA without modification of antioxidant enzyme activities. We suggest that mutations in mtDNA affecting complex I activity may result in oxidative cellular damage, and reinforce the possible role of ROS-mediated mechanisms participating in some mtDNA-related disorders.
authors.affiliation: Centre d'Investigacions en Bioquimica i Biologia Molecular, Hospital Vall d'Hebron, Passeig Vall d'Hebron 119-129, 08035 Barcelona, Spain.
authors.forename: Ricardo
authors.initials: R
authors.lastname: Gonzalo
authors.forename: Elena
authors.initials: E
authors.lastname: Garcia-Arumi
authors.forename: David
authors.initials: D
authors.lastname: Llige
authors.forename: Ramon
authors.initials: R
authors.lastname: Marti
authors.forename: Abelardo
authors.initials: A
authors.lastname: Solano
authors.forename: Julio
authors.initials: J
authors.lastname: Montoya
authors.forename: Joaquín
authors.initials: J
authors.lastname: Arenas
authors.forename: Antonio L
authors.initials: AL
authors.lastname: Andreu
mesh_terms: D004272:DNA, Mitochondrial / Q000235:genetics*; D006801:Humans; D008055:Lipids / Q000502:physiology; D008928:Mitochondria / Q000235:genetics / Q000378:metabolism*; D009154:Mutation; D009245:NADH Dehydrogenase / Q000737:chemistry / Q000235:genetics* / Q000378:metabolism; D010084:Oxidation-Reduction; D018384:Oxidative Stress / Q000235:genetics; D021122:Protein Subunits / Q000737:chemistry / Q000235:genetics; D017382:Reactive Oxygen Species / Q000378:metabolism*
chemical_list: D004272:DNA, Mitochondrial; D008055:Lipids; D021122:Protein Subunits; D017382:Reactive Oxygen Species; C507225:MT-ND6 protein, human; D009245:NADH Dehydrogenase