Article
Assembly defects induce oxidative stress in inherited mitochondrial complex I deficiency.
The international journal of biochemistry & cell biology - 1 Aug 2015
Leman Géraldine, Gueguen Naïg, Desquiret-Dumas Valérie, Kane Mariame Selma, Wettervald Céline, Chupin Stéphanie, Chevrollier Arnaud, Lebre Anne-Sophie, Bonnefont Jean-Paul, Barth Magalie, Amati-Bonneau Patrizia, Verny Christophe, Henrion Daniel, Bonneau Dominique, Reynier Pascal, Procaccio Vincent
Abstract excerpt
Complex I (CI) deficiency is the most common respiratory chain defect representing more than 30% of mitochondrial diseases. CI is an L-shaped multi-subunit complex with a peripheral arm protruding into the mitochondrial matrix and a membrane arm. CI sequentially assembled into main assembly intermediates: the P (pumping), Q (Quinone) and N (NADH dehydrogenase) modules. In this study, we analyzed 11 fibroblast...
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