COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness SF Heeringa, G Chernin, M Chaki, W Zhou, AJ Sloan, Z Ji, LX Xie, ... The Journal of clinical investigation 121 (5), 2013-2024, 2011 | 436 | 2011 |
Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis JM López-Martín, L Salviati, E Trevisson, G Montini, S DiMauro, C Quinzii, ... Human molecular genetics 16 (9), 1091-1097, 2007 | 158 | 2007 |
Biosynthesis of ascorbic acid in kidney bean. L-Galactono-γ-lactone dehydrogenase is an intrinsic protein located at the mitochondrial inner membrane E Siendones, JA González-Reyes, C Santos-Ocana, P Navas, F Córdoba Plant Physiology 120 (3), 907-912, 1999 | 153 | 1999 |
Is coenzyme Q a key factor in aging? G López-Lluch, JC Rodríguez-Aguilera, C Santos-Ocaña, P Navas Mechanisms of ageing and development 131 (4), 225-235, 2010 | 152 | 2010 |
Haploinsufficiency of COQ4 causes coenzyme Q10 deficiency L Salviati, E Trevisson, MAR Hernandez, A Casarin, V Pertegato, ... Journal of medical genetics 49 (3), 187-191, 2012 | 130 | 2012 |
Uptake of exogenous coenzyme Q and transport to mitochondria is required for bc1 complex stability in yeast coq mutants C Santos-Ocaña, TQ Do, CF Clarke, S Padilla, P Navas Journal of Biological Chemistry 277 (13), 10973-10981, 2002 | 130 | 2002 |
Genetic or other causation should not change the clinical diagnosis of cerebral palsy AH MacLennan, S Lewis, A Moreno-De-Luca, M Fahey, RJ Leventer, ... Journal of child neurology 34 (8), 472-476, 2019 | 111 | 2019 |
Effect of vanillic acid on COQ6 mutants identified in patients with coenzyme Q10 deficiency M Doimo, E Trevisson, R Airik, M Bergdoll, C Santos-Ocaña, ... Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1842 (1), 1-6, 2014 | 84 | 2014 |
Coenzyme Q: molecular mechanisms in health and disease VE Kagan, PJ Quinn CRC Press, 2000 | 83 | 2000 |
Demethoxy-Q, an intermediate of coenzyme Q biosynthesis, fails to support respiration in Saccharomyces cerevisiae and lacks antioxidant activity S Padilla, T Jonassen, MA Jiménez-Hidalgo, DJM Fernández-Ayala, ... Journal of Biological Chemistry 279 (25), 25995-26004, 2004 | 80 | 2004 |
Mitochondrial responsibility in ageing process: innocent, suspect or guilty G López-Lluch, C Santos-Ocaña, JA Sánchez-Alcázar, ... Biogerontology 16, 599-620, 2015 | 73 | 2015 |
Functional characterization of human COQ4, a gene required for Coenzyme Q10 biosynthesis A Casarin, JC Jimenez-Ortega, E Trevisson, V Pertegato, M Doimo, ... Biochemical and biophysical research communications 372 (1), 35-39, 2008 | 72 | 2008 |
Genetic evidence for coenzyme Q requirement in plasma membrane electron transport C Santos–Ocaña, JM Villalba, F Córdoba, S Padilla, FL Crane, CF Clarke, ... Journal of bioenergetics and biomembranes 30, 465-475, 1998 | 68 | 1998 |
Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis TPT Nguyen, A Casarin, MA Desbats, M Doimo, E Trevisson, ... Biochimica et Biophysica Acta (BBA)-Molecular and Cell Biology of Lipids …, 2014 | 65 | 2014 |
Structural basis of mitochondrial dysfunction in response to cytochrome c phosphorylation at tyrosine 48 B Moreno-Beltran, A Guerra-Castellano, A Diaz-Quintana, R Del Conte, ... Proceedings of the National Academy of Sciences 114 (15), E3041-E3050, 2017 | 64 | 2017 |
Coenzyme Q6 and iron reduction are responsible for the extracellular ascorbate stabilization at the plasma membrane of Saccharomyces cerevisiae C Santos-Ocana, F Córdoba, FL Crane, CF Clarke, P Navas Journal of Biological Chemistry 273 (14), 8099-8105, 1998 | 63 | 1998 |
Analysis of the sorting signals directing NADH-cytochrome b5 reductase to two locations within yeast mitochondria V Haucke, CS Ocana, A Hönlinger, K Tokatlidis, N Pfanner, G Schatz Molecular and cellular biology, 1997 | 59 | 1997 |
Hydroxylation of demethoxy-Q6 constitutes a control point in yeast coenzyme Q6 biosynthesis S Padilla, UC Tran, M Jiménez-Hidalgo, JM López-Martín, ... Cellular and Molecular Life Sciences 66, 173-186, 2009 | 58 | 2009 |
The phosphatase Ptc7 induces coenzyme Q biosynthesis by activating the hydroxylase Coq7 in yeast A Martín-Montalvo, I González-Mariscal, T Pomares-Viciana, ... Journal of Biological Chemistry 288 (39), 28126-28137, 2013 | 54 | 2013 |
Regulation of coenzyme Q biosynthesis in yeast: a new complex in the block I González‐Mariscal, E García‐Testón, S Padilla, A Martín‐Montalvo, ... IUBMB life 66 (2), 63-70, 2014 | 49 | 2014 |