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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
biochemistry, genetics and molecular biology
Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ
10
deficiency
FASEB Journal, Volume 24, No. 10, Year 2010
Notification
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Description
Coenzyme Q10 (CoQ10) is essential for electron transport in the mitochondrial respiratory chain and antioxidant defense. The relative importance of respiratory chain defects, ROS production, and apoptosis in the pathogenesis of CoQ10 deficiency is unknown. We determined previously that severe CoQ10 deficiency in cultured skin fibroblasts harboring COQ2 and PDSS2 mutations produces divergent alterations of bioenergetics and oxidative stress. Here, to better understand the pathogenesis of CoQ10 deficiency, we have characterized the effects of varying severities of CoQ10 deficiency on ROS production and mitochondrial bioenergetics in cells harboring genetic defects of CoQ10 biosynthesis. Levels of CoQ10 seem to correlate with ROS production; 10-15% and >60% residual CoQ10 are not associated with significant ROS production, whereas 30-50% residual CoQ10 is accompanied by increased ROS production and cell death. Our results confirm that varying degrees of CoQ10 deficiency cause variable defects of ATP synthesis and oxidative stress. These findings may lead to more rational therapeutic strategies for CoQ10 deficiency. © FASEB.
Authors & Co-Authors
Quinzii, Catarina Maria
United States, New York
Columbia University Irving Medical Center
López, Luís Carlos
United States, New York
Columbia University Irving Medical Center
Gilkerson, Robert W.
United States, New York
Columbia University Irving Medical Center
Dorado, Beatriz
United States, New York
Columbia University Irving Medical Center
Coku, Jorida
United States, New York
Columbia University Irving Medical Center
Naini, Ali B.
United States, New York
Columbia University Irving Medical Center
Lagier-Tourenne, Clotilde
France, Illkirch-graffenstaden
Igbmc Institut de Genetique et de Biologie Moleculaire et Cellulaire
Schuelke, Markus
Germany, Berlin
Charité – Universitätsmedizin Berlin
Salviati, Leonardo
Italy, Padua
Università Degli Studi Di Padova
Carrozzo, Rosalba
Italy, Rome
Irccs Ospedale Pediatrico Bambino Gesù
Santorelli, Filippo Maria
Italy, Rome
Irccs Ospedale Pediatrico Bambino Gesù
Rahman, Shamima
United Kingdom, London
University College London
Tazir, Mériem
Algeria, Algiers
Centre Hospitalo-universitaire de Mustapha Bacha
Kœnig, Michel
France, Illkirch-graffenstaden
Igbmc Institut de Genetique et de Biologie Moleculaire et Cellulaire
DiMauro, Salvatore
United States, New York
Columbia University Irving Medical Center
Hirano, Michio
United States, New York
Columbia University Irving Medical Center
Statistics
Citations: 142
Authors: 16
Affiliations: 7
Identifiers
Doi:
10.1096/fj.09-152728
ISSN:
08926638
Research Areas
Cancer
Genetics And Genomics