The antioxidant Trolox restores mitochondrial membrane potential and Ca2+-stimulated ATP production in human complex I deficiency
暂无分享,去创建一个
[1] H. Rolleston. The Cambridge Medical School: Department of Biochemistry , 2009 .
[2] J. Smeitink,et al. Mitigation of NADH: ubiquinone oxidoreductase deficiency by chronic Trolox treatment. , 2008, Biochimica et biophysica acta.
[3] J. Smeitink,et al. Mitochondrial Ca2+ homeostasis in human NADH:ubiquinone oxidoreductase deficiency. , 2008, Cell calcium.
[4] R. Rodenburg,et al. NDUFA2 complex I mutation leads to Leigh disease. , 2008, American journal of human genetics.
[5] Ann Saada,et al. Mitochondrial complex I deficiency caused by a deleterious NDUFA11 mutation , 2008, Annals of neurology.
[6] J. Smeitink,et al. Life cell quantification of mitochondrial membrane potential at the single organelle level , 2008, Cytometry. Part A : the journal of the International Society for Analytical Cytology.
[7] Ann Saada,et al. C6ORF66 is an assembly factor of mitochondrial complex I. , 2008, American journal of human genetics.
[8] R. Wanders,et al. Phytanic acid impairs mitochondrial respiration through protonophoric action , 2007, Cellular and Molecular Life Sciences.
[9] J. Smeitink,et al. Mitochondrial and cytosolic thiol redox state are not detectably altered in isolated human NADH:ubiquinone oxidoreductase deficiency. , 2007, Biochimica et biophysica acta.
[10] J. Silke,et al. Human CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause disease , 2007, The EMBO journal.
[11] J. Smeitink,et al. Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology? , 2007, American journal of physiology. Cell physiology.
[12] J. Smeitink,et al. Superoxide production is inversely related to complex I activity in inherited complex I deficiency. , 2007, Biochimica et biophysica acta.
[13] John E. Walker,et al. Bovine Complex I Is a Complex of 45 Different Subunits* , 2006, Journal of Biological Chemistry.
[14] Dimphy Zeegers,et al. Ca2+-mobilizing agonists increase mitochondrial ATP production to accelerate cytosolic Ca2+ removal: aberrations in human complex I deficiency. , 2006, American journal of physiology. Cell physiology.
[15] A. Leusink,et al. Decreased agonist-stimulated mitochondrial ATP production caused by a pathological reduction in endoplasmic reticulum calcium content in human complex I deficiency. , 2006, Biochimica et biophysica acta.
[16] Massimo Zeviani,et al. Mitochondrial medicine: a metabolic perspective on the pathology of oxidative phosphorylation disorders. , 2006, Cell metabolism.
[17] E. Shoubridge,et al. A molecular chaperone for mitochondrial complex I assembly is mutated in a progressive encephalopathy. , 2005, The Journal of clinical investigation.
[18] P. Brookes,et al. Mitochondrial H(+) leak and ROS generation: an odd couple. , 2005, Free radical biology & medicine.
[19] G. Rutter,et al. Inhibition of Mitochondrial Na (cid:1) -Ca 2 (cid:1) Exchange Restores Agonist-induced ATP Production and Ca 2 (cid:1) Handling in Human Complex I Deficiency* , 2004 .
[20] Robert W. Taylor,et al. NDUFS6 mutations are a novel cause of lethal neonatal mitochondrial complex I deficiency. , 2004, The Journal of clinical investigation.
[21] M. Duchen. Mitochondria in health and disease: perspectives on a new mitochondrial biology. , 2004, Molecular aspects of medicine.
[22] J. Smeitink,et al. Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency. , 2004, Human molecular genetics.
[23] A. J. Lambert,et al. Mitochondrial superoxide and aging: uncoupling-protein activity and superoxide production. , 2004, Biochemical Society symposium.
[24] P. Bénit,et al. Mutant NDUFS3 subunit of mitochondrial complex I causes Leigh syndrome , 2004, Journal of Medical Genetics.
[25] P. Bénit,et al. Mutant NDUFV2 subunit of mitochondrial complex I causes early onset hypertrophic cardiomyopathy and encephalopathy , 2003, Human mutation.
[26] Robin A. J. Smith,et al. Superoxide Activates Mitochondrial Uncoupling Protein 2 from the Matrix Side , 2002, The Journal of Biological Chemistry.
[27] Hui Zhang,et al. Oxidative stress increases internal calcium stores and reduces a key mitochondrial enzyme. , 2002, Biochimica et biophysica acta.
[28] P. Bénit,et al. Large-scale deletion and point mutations of the nuclear NDUFV1 and NDUFS1 genes in mitochondrial complex I deficiency. , 2001, American journal of human genetics.
[29] J. Smeitink,et al. Human NADH:Ubiquinone Oxidoreductase , 2001, Journal of bioenergetics and biomembranes.
[30] S. Dimauro,et al. The genetics and pathology of oxidative phosphorylation , 2001, Nature Reviews Genetics.
[31] P. Barth,et al. Leigh syndrome associated with a mutation in the NDUFS7 (PSST) nuclear encoded subunit of complex I , 1999, Annals of neurology.
[32] E. Mariman,et al. Mutant NDUFV1 subunit of mitochondrial complex I causes leukodystrophy and myoclonic epilepsy , 1999, Nature Genetics.
[33] B. Hamel,et al. The first nuclear-encoded complex I mutation in a patient with Leigh syndrome. , 1998, American journal of human genetics.
[34] S. Heales,et al. Peroxynitrite and Brain Mitochondria: Evidence for Increased Proton Leak , 1998, Journal of neurochemistry.
[35] E. Mariman,et al. Demonstration of a new pathogenic mutation in human complex I deficiency: a 5-bp duplication in the nuclear gene encoding the 18-kD (AQDQ) subunit. , 1998, American journal of human genetics.
[36] R Marsault,et al. Transfected Aequorin in the Measurement of Cytosolic Ca2+ Concentration ([Ca2+]c) , 1995, The Journal of Biological Chemistry.
[37] G L MADDOX,et al. A critical evaluation , 2012 .