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International Journal of Cancer|October 26, 2007
A cancer-predisposing "hot spot" mutation of the fumarase gene creates a dominant negative proteinAnnalisa Lorenzato, Martina Olivero, Mario Perro, et al.
Plos One|January 23, 2009
Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxiaVincent Paupe, Emmanuel P Dassa, Sergio Goncalves, et al.
Molecular Genetics and Metabolism|October 3, 2002
Coenzyme Q(10) and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefitsVanna Geromel, Niklas Darin, Dominique Chrétien, et al.
European Journal of Biochemistry|August 20, 2004
Processing of the 24 kDa subunit mitochondrial import signal is not required for assembly of functional complex I in Yarrowia lipolyticaStefan Kerscher, Paule Bénit, Albina Abdrakhmanova, et al.
European Journal of Haematology|July 22, 2006
Different pathophysiological mechanisms of intramitochondrial iron accumulation in acquired and congenital sideroblastic anemia caused by mitochondrial DNA deletionThomas Matthes, Pierre Rustin, Hedwige Trachsel, et al.
The Journal of Biological Chemistry|September 26, 2012
Glucose modulates respiratory complex I activity in response to acute mitochondrial dysfunctionGiuseppe Cannino, Riyad El-Khoury, Marja Pirinen, et al.
Free Radical Biology & Medicine|April 21, 2016
Expression of the alternative oxidase mitigates beta-amyloid production and toxicity in model systemsRiyad El-Khoury, Eveliina Kaulio, Katariina A Lassila, et al.
Human Molecular Genetics|March 19, 2004
Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxiaHervé Seznec, Delphine Simon, Laurent Monassier, et al.
Biomedicines|July 27, 2022
Succinate Dehydrogenase, Succinate, and Superoxides: A Genetic, Epigenetic, Metabolic, Environmental Explosive CrossroadPaule Bénit, Judith Goncalves, Riyad El Khoury, et al.
Clinical Science (London, England : 1979)|February 6, 2016
Mitochondrial cytochrome c oxidase deficiencyMalgorzata Rak, Paule Bénit, Dominique Chrétien, et al.
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