New insights into structure and function of mitochondria and their role in aging and disease

Antioxidants and Redox Signaling

Volumn 8, Issue 3-4, 2006, Pages 417-437

  Lenaz, Giorgio ^a   Baracca, Alessandra ^a   Fato, Romana ^a   Genova, Maria Luisa ^a   Solaini, Giancarlo ^a  

^a UNIVERSITY OF BOLOGNA   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

CELL NUCLEUS DNA; MITOCHONDRIAL DNA; OXYGEN RADICAL; REACTIVE OXYGEN METABOLITE; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE DEHYDROGENASE (UBIQUINONE); SUPEROXIDE;

AGING; CELL AGING; DISORDERS OF MITOCHONDRIAL FUNCTIONS; GENE DELETION; GENE MUTATION; GENETIC POLYMORPHISM; HUMAN; LEBER HEREDITARY OPTIC NEUROPATHY; MELAS SYNDROME; MITOCHONDRION; MOLECULAR MODEL; NARP SYNDROME; NONHUMAN; OXIDATIVE PHOSPHORYLATION; PRIORITY JOURNAL; RESPIRATORY CHAIN; REVIEW; SIGNAL TRANSDUCTION; SOMATIC MUTATION; STRUCTURAL GENE; THEORETICAL MODEL;

AGING; ANIMALS; DNA, MITOCHONDRIAL; ELECTRON TRANSPORT; HUMANS; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MODELS, BIOLOGICAL; MUTATION; OXYGEN; PHOSPHORYLATION; REACTIVE OXYGEN SPECIES; SUPEROXIDES;

EID: 33646684516     PISSN: 15230864     EISSN: None     Source Type: Journal    
DOI: 10.1089/ars.2006.8.417     Document Type: Review

References (262)

1. Allen RG, Keogh BP, Tresini M, Gerhrad GS, Volker C, Pignolo RJ, Horton J, and Cristofalo VJ. Development and age-associated differences in electron transport potential and consequences for oxidant generation. J Biol Chem 272: 24805-24812, 1997.
2. Anson RM and Hansford RG. Mitochondrial influence on aging rate in Caenorhabditis elegans. Aging Cell 3: 29-34, 2003.
3. Attardi G, Enriquez JA, and Cabezas-Herrera J. Intermitochondrial complementation of mtDNA mutations and nuclear context. Nat Genet 30: 360, 2002.
4. Balazy M and Nigam S. Aging, lipid modifications and phospholipases-new concepts. Ageing Res Rev 2: 191-209, 2003.
5. Baracca A, Barogi S, Carelli V, Lenaz G, and Solaini G. Catalytic activities of mitochondrial ATP synthase in patients with mitochondrial DNA T8993G mutation in the ATPase 6 gene encoding subunit a. J Biol Chem 275: 4177-4182, 2000.
6. Baracca A, Sgarbi G, Solaini G, and Lenaz G. Rhodamine 123 as a probe of mitochondrial membrane potential: evaluation of proton flux through F(0) during ATP synthesis. Biochim Biophys Acta 1606: 137-146, 2003.
7. Baracca A, Solaini G, Sgarbi G, Lenaz G, Baruzzi A, Schapira AHV, Martinuzzi A, and Carelli V. Severe impairment of complex I-driven ATP synthesis in Leber's hereditary optic neuropathy cybrids. Arch Neurol 62: 730-736, 2005.
8. Barazzoni R, Short KR, and Sreekumaran KN. Effects of aging on mitochondrial DNA copy number and cytochrome c oxidase gene expression in rat skeletal muscle, liver, and heart. J Biol Chem 275: 3343-3347, 2000.
9. Barja G. Aging in vertebrates, and the effect of caloric restriction: a mitochondrial free radical production-DNA damage mechanism? Biol Rev 79: 235-251, 2004.
10. Barja G and Herrero A. Oxidative damage to mitochondrial DNA is inversely related to maximum life span in the heart and brain of mammals. FASEB J 14: 312-318, 2000.
11. Barogi S, Baracca A, Cavazzoni M, Parenti Castelli G, and Lenaz G. Effect of the oxidative stress induced by adriamycin on rat hepatocyte bioenergetics during ageing. Mech Ageing Dev 113: 1-21, 2000.
12. Barrientos A, Casademont J, Cardellach F, Estivill X, Urbano-Marquez A, and Nunes V. Reduced steady-state levels of mitochondrial RNA and increased mitochondrial DNA amount in human brain with aging. Brain Res Mol Brain Res 52: 284-289, 1997.
13. Barrientos A and Moraes CT. Titrating the effects of mitochondrial complex I impairment in the cell physiology. J Biol Chem 274: 16188-16197, 1999.
14. Beal MF. Does impairment of energy metabolism result in excitotoxic neuronal death in neurodegenerative illnesses? Am Neurol 31: 119-130, 1992.
15. Beckman KB and Ames BN. The free radical theory of aging matures. Physiol Rev 78: 547-581, 1998.
16. Bernardi P. Mitochondrial transport of cations: channels, exchangers, and permeability transition. Physiol Rev 79: 1127-1155, 1999.
17. Bejma J and Ji LL. Aging and acute exercise enhance free radical generation in rat skeletal muscle. J Appl Physiol 87: 465-470, 1999.
18. Bianchi C, Fato R, Genova ML, Parenti Castelli G, and Lenaz G. Structural and functional organization of Complex I in the mitochondrial respiratory chain. Biofactors 18: 3-9, 2003.
19. Bianchi C, Genova ML, Parenti Castelli G, and Lenaz G. The mitochondrial respiratory chain is partially organized in a supercomplex assembly: kinetic evidence using flux control analysis. J Biol Chem 279: 36562-36569, 2004.
20. Bindoli A, Deeble DJ, Rigobello MP, and Galzigna L. Direct and respiratory chain-mediated redox cycling of adrenochrome. Biochim Biophys Acta 1016: 349-356, 1990.
21. Borras C, Sastre J, Garcia-Sala D, Lloret A, Pallardo FV, and Vina J. Mitochondria from females exhibit higher antioxidant gene expression and lower oxidative damage than males. Free Radic Biol Med 34: 546-552, 2003.
22. Boumans H, Grivell LA, and Berden JA. The respiratory chain in yeast behaves as a single functional unit. J Biol Chem 273: 4872-4877, 1998.
23. Boveris A, Oshino N, and Chance B. The cellular production of hydrogen peroxide. Biochem J 128: 617-630, 1972.
24. Brand MD. Uncoupling to survive? The role of mitochondrial inefficiency in ageing. Exp Gerontol 35: 811-820, 2000.
25. Brand MD, Affourtit C, Esteves TC, Green K, Lambert AJ, Miwa S, Pakay JL, and Parker N. Mitochondrial superoxide: production, biological effects, and activation of uncoupling proteins. Free Radic Biol Med 37: 755-767, 2004.
26. Brandt U. Proton-translocation by membrane-bound NADH:ubiquinone- oxidoreductase (complex I) through redox-gated ligand conduction. Biochim Biophys Acta 1318: 79-91, 1997.
27. Brandt U, Kerscher S, Drose S, Zwicker K, and Zickermann V. Proton pumping by NADH:ubiquinone oxidoreductase. A redox driven conformational change mechanism? FEBS Lett 545: 9-17, 2003.
28. Brown MD. The enigmatic relationship between mitochondrial dysfunction and Leber's hereditary optic neuropathy. J Neurol Sci 165: 1-5, 1999.
29. Brown WM, Gorge M, and Wilson AC. Rapid evolution of animal mitochondrial DNA. Proc Natl Acad Sci USA 76: 1967-1971, 1979.
30. Butow RA. Cellular responses to mitochondrial dysfunction: it's not always downhill Cell Death Differ 9: 1043-1045, 2002.
31. Butow RA and Avadhani NG. Mitochondrial signaling: the retrograde response. Mol Cell 14: 1-15, 2004.
32. Byrne E, Dennet X, and Trounce I. Oxidative energy failure in post-mitotic cells: a major factor in senescence. Rev Neurol 147: 532-535, 1991.
33. Calabrese V, Scapagnini G, Colombrita C, Ravagna A, Pennini G, Giuffrida Stella AM, Galli F, and Butterfield DA. Redox regulation of heat shock protein expression in aging and neurodegenerative disorders associated with oxidative stress: a nutritional approach. Amino Acids 25: 437-444, 2003.
34. Calabrese V, Scapagnini G, Giuffrida Stella AM, Bates TE, and Clark JB. Mitochondrial involvement in brain function and dysfunction: relevance to aging, neurodegenerative disorders and longevity. Neurochem Res 26: 739-764, 2001.
35. Calabrese V, Scapagnini G, Ravagna A, Colombrita C, Spadaro F, Butterfield DA, and Giuffrida Stella AM. Increased expression of heat shock proteins in rat brain during aging: relationship with mitochondrial function and glutathione redox state. Mech Ageing Dev 125: 325-335, 2004.
36. Camougrand N and Rigoulet M. Aging and oxidative stress: studies of some genes involved both in aging and in response to oxidative stress. Respir Physiol 128: 393-401, 2001.
37. 2 release during aging in oxidative and glycolytic muscles in rats. Mech Ageing Dev 125: 367-373, 2004.
38. nd ed., Schapira AHV, Di Mauro S., eds. Boston, MA: Butterworth-Heinemann, 2002, 115-142.
39. Carelli V, Baracca A, Barogi S, Pallotti F, Valentino ML, Montagna P, Zeviani M, Pini A, Lenaz G, Baruzzi A, and Solaini G. Biochemical-clinical correlation in patients with different loads of the mitochondrial DNA T8993G mutation. Arch Neurol 59: 264-270, 2002.
40. Carelli V, Ghelli A, Bucchi L, Montagna P, De Negri A, Lezzi V, Carducci C, Lenaz G, Lugaresi E, and Degli Esposti M. Biochemical features of mtDNA 14484 (ND6/M64V) point mutation associated with Leber's hereditary optic neuropathy. Ann Neurol 45: 320-328, 1999.
41. Carelli V, Ghelli A, Ratta M, Bacchilega E, Sangiorgi S, Mancini R, Leuzzi V, Cortelli P, Montagna P, Lugaresi E, and Degli Esposti M. Leber's hereditary optic neuropathy: biochemical effect of 11778/ND4 and 3460/ND1 mutations and correlation with the mitochondrial genotype. Neurology 48: 1623-1632, 1997.
42. Carelli V, Ross-Cisneros FN, and Sadun AA. Optic nerve degeneration and mitochondrial dysfunction: genetic and acquired optic neuropathies. Neurochem Int 40: 573-584, 2002.
43. Carelli V, Rugolo M, Sgarbi G, Ghelli A, Zanna C, Baracca A, Lenaz G, Napoli E, Martinuzzi A, and Solaini G. Bioenergetics shapes cellular death pathways in Leber's hereditary optic neuropathy: a model of mitochondrial neurodegeneration. Biochim Biophys Acta 1658: 172-179, 2004.
44. Carroll J, Fearnley IM, Shannon RG, Hirst J, and Walker JE. The nuclear encoded subunits of complex I from bovine heart mitochondria. Mol Cell Proteomics 2: 117-126, 2003.
45. Casteilla L, Rigoulet M, and Pénicaud L. Mitochondrial ROS metabolism: modulation by uncoupling proteins. IUBMB Life 52: 181-188, 2001.
46. Cavazzoni M, Barogi S, Baracca A, Parenti Castelli G, and Lenaz G. The effect of aging and an oxidative stress on peroxide levels and the mitochondrial membrane potential in isolated rat hepatocytes. FEBS Lett 440: 53-56, 1999.
47. Chen C, Ko YH, Delannoy M, Ludtke SJ, Chiu W, and Pedersen PL. Mitochondrial ATP synthasome: three-dimensional structure by electron microscopy of the ATP synthase in complex formation with carriers for Pi and ADP/ATP. J Biol Chem 279: 31761-31768, 2004.
48. Chen Q, Vazquez EJ, Moghaddas S, Hoppel CL, and Lesnefsky E. Production of reactive oxygen species by mitochondria: central role of complex III. J Biol Chem 278: 36027-36031, 2003.
49. Chomyn A, Mariottini P, Cleeter MWJ, Ragan CI, Matsuno-Yagi A, Hatefi Y, Doolite RF, and Attardi G. Six unidentified reading frames of human mitochondrial DNA encode components of the respiratory-chain NADH dehydrogenase. Nature 314: 592-597, 1985.
50. Chung HY, Kim HJ, Kim KW, Choi JS, and Yu BP. Molecular inflammation hypothesis of aging based on the anti-aging mechanism of calorie restriction. Microsc Res Tech 59: 264-272, 2002.
51. Cock HR, Cooper JM, Schapira AHV. The 14484 ND6 mtDNA mutation in Leber hereditary optic neuropathy does not affect fibroblast complex I activity. Am J Hum Genet 57: 1501-1502, 1995.
52. Cooper TM, Mockett RJ, Sohal BH, Sohal RS, and Orr WC. Effect of caloric restriction on life span of the housefly, Musca domestica. FASEB J 18: 1591-1593, 2004.
53. Cortopassi GA. Fixation of deleterious alleles, evolution and human aging. Mech Ageing Dev 123: 851-855, 2002.
54. Cortopassi GA and Wang E. Modelling the effects of age-related mtDNA mutation accumulation; complex I deficiency, superoxide and cell death. Biochim Biophys Acta 1271, 171-176, 1995.
55. Cutler RG. Peroxide-producing potential of tissues: inverse correlation with longevity of mammalian species. Proc Natl Acad Sci USA 82: 4798-4802, 1985.
56. D'Aurelio M, Gajewski CD, Lin MT, Mauck WM, Shao LZ, Lenaz G, Moraes CT, and Manfredi G. Heterologous mitochondrial DNA recombination in human cells. Hum Mol Genet 13: 3171-3179, 2004.
57. De Benedictis G, Rose G, Carrieri G, De Luca M, Falcone E, Passarino G, Bonafè M, Monti D, Baggio G, Bertolini S, Mari D, Mattace R, and Franceschi C. Mitochondrial DNA inherited variants are associated with successful aging and longevity in humans. FASEB J 13: 1532-1536, 1999.
58. De Grey AD. The reductive hotspot hypothesis of mammalian aging: membrane metabolism magnifies mutant mitochondrial mischief. Eur J Biochem 269: 2003-2009, 2002.
59. De Groot J. The AGE of the matrix: chemistry, consequence and cure. Curr Opinion Pharmacol 4: 301-305, 2004.
60. Deleonardi G, Biondi A, D'Aurelio M, Merlo Pich M, Stankov K, Falasca A, Formaggini G, Bovina C, Romeo G, and Lenaz G. Plasma membrane oxidoreductase activity in cultured cells in relation to mitochondrial function and oxidative stress. BioFactors 20: 251-258, 2004.
61. De Magalhaes JP. From cells to ageing: a review of models and mechanisms of cellular senescence and their impact on human ageing. Exp Cell Res 300: 1-10, 2004.
62. De Meirleir L, Seneca S, Lissens W, Schoentjes E, and Desprechins B. Bilateral striatal necrosis with a novel point mutation in the mitochondrial ATPase 6 gene. Pediatr Neurol 13: 242-246, 1995.
63. Degli Esposti M. Inhibitors of NADH-ubiquinone reductase: an overview. Biochim Biophys Acta 1364: 222-235, 1998.
64. Degli Esposti M, Carelli V, Ghelli A, Ratta M, Crimi M, Sangiorgi S, Montagna P, Lugaresi E, and Cortelli P. Functional alterations of the mitochondrially encoded ND4 subunit associated with Leber's hereditary optic neuropathy. FEBS Lett 352: 375-379, 1994.
65. Di Mauro S. Mitochondrial diseases. Biochim Biophys Acta 1658: 80-88, 2004.
66. Di Mauro S and Schon EA. Mitochondrial respiratory-chain diseases. N Engl J Med 348: 2656-2668, 2003.
67. Diplock AT. Antioxidants and disease prevention. Mol Aspects Med 15: 293-376, 1994.
68. Dizdaroglu M, Jaruga P, Birincioglu M, and Rodriguez H. Free radical-induced damage to DNA: mechanisms and measurement. Free Radic Biol Med 32: 1102-1115, 2002.
69. Djafarzadeh R, Kerscher S, Zwicker K, Radermacher M, Lindahl M, Schägger H, and Brandt U. Biophysical and structural characterization of proton-translocating NADH-dehydrogenase (complex I) from the strictly aerobic yeast Yarrowia lipolytica. Biochim Biophys Acta 1459: 230-238, 2000.
70. Drahota Z, Chowdhury SK, Floryk D, Mracek T, Wilhelm J, Rauchova H, Lenaz G, and Houstek J. Glycerophospate-dependent hydrogen peroxide production by brown adipose tissue mitochondria and its activation by ferricyanide. J Bioenerg Biomembr 34: 105-113, 2002.
71. Drew B, Phaneuf S, Dirks A, Selman C, Gredilla R, Lezza A, Barja G, and Leeuwenburgh C. Effects of aging and caloric restriction on mitochondrial energy production in gastrocnemius muscle and heart. Am J Physiol Regul Integr Comp Physiol 284: R474-R480, 2003.
72. Dudkina NV, Eubel H, Keegstra W, Boekema EJ, and Braun HP. Structure of mitochondrial supercomplex formed by respiratory-chain complexes I and III. Proc Natl Acad Sci USA 102: 3225-3229, 2005.
73. Dufour E and Larsson NG. Understanding aging: revealing order out of chaos. Biochim Biophys Acta 1658: 122-132, 2004.
74. Duval C, Negre-Salvayre A, Doglio A, Salvare R, Penicaud L, and Casteilla L. Increased reactive oxygen species production with antisense oligonucleotides directed against uncoupling protein 2 in murine endothelial cells. Biochem Cell Biol 80: 757-764, 2002.
75. Echtay KS, Murphy MP, Smith RA, Talbot DA, and Brand MD. Superoxide activates mitochondrial uncoupling protein 2 from the matrix side. Studies using targeted antioxidants. J Biol Chem 277: 47129-47135, 2002.
76. Effros RB. From Hayflick to Walford: the role of T cell replicative senescence in human aging. Exp Gerontol 39: 885-890, 2004.
77. Enriquez JA, Cabezas-Herrera J, Balona-Bafaluy MP, and Attardi G. Very rare complementation between mitochondria carrying different mitochondrial DNA mutations points to intrinsic genetic autonomy of the organelles in cultured human cells. J Biol Chem 275: 11207-11215, 2000.
78. Ernster L. Lipid peroxidation in biological membranes: mechanisms and implications. In: Active Oxygen, Lipid Peroxides and Antioxidants, Yagi K, ed. Boca Raton: CRC Press, 1993, 1-38.
79. Ernster L and Dallner G. Biochemical, physiological and medical aspects of ubiquinone function. Biochim Biophys Acta 1271: 195-204, 1995.
80. Esposito LA, Kokoszka JE, Waymire KG, Cottrell B, MacGregor GR, and Wallace DC. Mitochondrial oxidative stress in mice lacking the glutathione peroxidase-1 gene. Free Radic Biol Med 28: 754-766, 2000.
81. Eubel H, Jansch L, and Braun HP. New insights into the respiratory chain of plant mitochondria. Supercomplexes and a unique composition of complex II. Plant Physiol 133: 274-286, 2003.
82. Fato R, Cavazzoni M, Castelluccio C, Parenti Castelli G, Palmer G, Degli Esposti M, and Lenaz G. Steady-state kinetics of ubiquinol-cytochrome c reductase in bovine heart submitochondrial particles: diffusional effects. Biochem J 290: 225-236, 1993.
83. Gadaleta MN, Petruzzella V, Renis M, Fracasso F, and Cantatore P. Reduced transcription of mitochondrial DNA in the senescent rat. Tissue dependence and effect of L-carnitine. Eur J Biochem 187: 501-506, 1990.
84. Gadaleta MN, Rainaldi G, Lezza AMS, Milella F, Fracasso F, and Cantatore P. Mitochondrial DNA copy number and mitochondrial DNA deletion in adult and senescent rats. Mutat Res 275: 181-193, 1992.
85. Garcia-Riuz C, Colell A, Paris R, and Fernandez-Checa JC. Direct interaction of GD3 ganglioside with mitochondria generates reactive oxygen species followed by mitochondrial permeability transition, cytochrome c release, and caspase activation. FASEB J 14: 847-858, 2000.
86. Genova ML, Bovina C, Marchetti M, Pallotti F, Tietz C, Biagini G, Pugnaloni A, Viticchi C, Gorini A, Villa RF, and Lenaz G. Decrease of rotenone inhibition is a sensitive parameter of complex I damage in brain non-synaptic mitochondria of aged rats. FEBS Lett 410: 467-469, 1997.
87. Genova ML, Merlo Pich M, Biondi A, Bernacchia A, Falasca A, Bovina C, Formiggini G, Parenti Castelli G, and Lenaz G. Mitochondrial production of oxygen radical species and the role of Coenzyme Q as an antioxidant. Exp Biol Med (Maywood) 228: 506-513, 2003.
88. Genova ML, Ventura B, Giuliano G, Bovina C, Formiggini G, Parenti Castelli G, and Lenaz G. The site of production of superoxide radical in mitochondrial Complex I is not a bound ubisemiquinone but presumably iron-sulfur cluster N2. FEBS Lett 505: 364-368, 2001.
89. Ghelli A, Zanna C, Porcelli AM, Schapira AHV, Martinuzzi A, Carelli V, and Rugolo M. Leber's hereditary optic neuropathy (LHON) pathogenic mutations induce mitochondrial-dependent apoptotic death in transmitochondrial cells incubated with galactose medium. J Biol Chem 278: 4145-4150, 2003.
90. Gilderson G, Salomonsson L, Aagard A, Gray J, Brzezinski P, and Hosler J. Subunit III of cytochrome c oxidase of Rhodobacter sphaeroides is required to maintain rapid proton uptake through the D pathway at physiologic pH. Biochemistry 42: 7400-7409, 2003.
91. Götz ME, Künig G, Riederer P, and Youdim MBH. Oxidative stress: free radical production in neural degeneration. Pharmacol Ther 63: 37-122, 1994.
92. Greco M, Villani G, Mazzucchelli F, Bresolin N, Papa S, and Attardi G. Marked aging-related decline in efficiency of oxidative phosphorylation in human skin fibroblasts. FASEB J 17: 1706-1708, 2004.
93. Hackenbrock CR, Chazotte B, and Gupte SS. The random collision model and a critical assessment of diffusion and collision in mitochondrial electron transport. J Bioenerg Biomembr 18: 331-368, 1986.
94. Hall DM, Sattler GL, Zhang HJ, Oberley LW, Pilot HC, and Kregel KC. Aging lowers steady-state antioxidant enzyme and stress protein expression in primary hepatocytes. J Gerontol A Biol Sci Med Sci 56: B259-B267, 2001.
95. Haltia T and Saraste MMW. Subunit III of cytochrome c oxidase is not involved in proton translocation: a site-directed mutagenesis study. EMBO J 10: 2015-2021, 1991.
96. Han D, Antunes F, Canali R, Rettori D, and Cadenas E. Voltage-dependent anion channels control the release of the superoxide anion from mitochondria to cytosol. J Biol Chem 278: 5557-5563, 2003.
97. H) which is capable of fully coupled oxidative phosphorylation. Biochim Biophys Acta 81: 214-222, 1964.
98. 2 formation by rat heart mitochondria on substrate availability and donor age. J Bioenerg Biomembr 29: 89-95, 1997.
99. Hanson BJ, Carrozzo R, Piemonte F, Tessa A, Robinson BH, and Capaldi RA. Cytochrome c oxidase-deficient patients have distinct subunit assembly profiles. J Biol Chem 276: 16296-16301, 2001.
100. Hayakawa M, Hattori K, Sugiyama S, and Ozawa T. Age-associated oxygen damage and mutations in mitochondrial DNA in human hearts. Biochem Biophys Res Commun 189: 979-985, 1992.
101. Hayakawa M, Katsumata K, Yoneda M, Tanaka M, Sugiyama S, and Ozawa T. Age-related extensive fragmentation of mitochondrial DNA into minicircles. Biochem Biophys Res Commun 226: 369-377, 1996.
102. Hayflick L. Aging, longevity, and immortality in vitro. Exp Gerontol 27: 363-368, 1992.
103. Hayflick L. Living forever and dying in the attempt. Exp Gerontol 28: 1231-1241, 2003.
104. Hekimi S and Guarente L. Genetics and the specificity of the aging process. Science 299: 1351-1354, 2003.
105. Helfand SL and Inouye SK. Rejuvenating views of the ageing process. Nat Rev Genet 3: 149-153, 2002.
106. Herrero A and Barja G. Localization of the site of oxygen radical generation inside the Complex I of heart and non-synaptic brain mammalian mitochondria. J Bioenerg Biomembr 32: 609-616, 2000.
107. Hoffbuhr KC, Davidson E, Filiano BA, Davidson M, Kennaway NG, and King MP. A pathogenic 15-base pair deletion in mitochondrial DNA-encoded cytochrome c oxidase subunit III results in the absence of functional cytochrome c oxidase. J Biol Chem 575: 13994-14003, 2000.
108. Hofhaus G, Berneburg M, Wulfert M, and Gattermann N. Live now-pay by ageing: high performance mitochondrial activity in youth and its age-related side effects. Exp Physiol 881: 167-174, 2003.
109. Hofhaus G and Gattermann N. Mitochondria harbouring mutant mtDNA-a cuckoo in the nest? Biol Chem 380: 871-877, 1999.
110. Holt J, Harding AE, Petty RKH, and Morgan-Hughes JA. A new mitochondrial disease associated with mitochondrial DNA heteroplasmy. Am J Hum Genet 46: 428-433, 1990.
111. Hosokawa M. A higher oxidative status accelerates senescence and aggravates age-dependent disorders in SAMP strains of mice. Mech Ageing Dev 123: 1553-1561, 2002.
112. Houstek J, Klement P, Hermanska J, Houstekova H, Van de Bogert C, and Zeman J. Altered properties of mitochondrial ATP-synthase in patients with a T→G mutation in the ATPase 6 (subunit a) gene at position 8993 of mtDNA. Biochim Biophys Acta 1271: 349-357, 1995.
113. Houstek J, Mracek T, Vojtiskova A, and Zeman J. Mitochondrial diseases and ATPase defects of nuclear origin. Biochim Biophys Acta 1658: 115-121, 2004.
114. Howell N, Bindoff LA, McCullough DA, Kubacka I, Poulton J, Mackey D, Taylor L, and Turnbull DM. Leber hereditary optic neuropathy: identification of the same mitochondrial ND1 mutation in six pedigrees. Am J Hum Genet 49: 939-950, 1991.
115. Hutter E, Unterluggauer H, Uberall F, Schramek H, and Jansen-Durr P. Replicative senescence of human fibroblasts: the role of Ras-dependent signaling and oxidative stress. Exp Gerontol 37: 1165-1174, 2002.
116. Ikeyama N, Kokkonen G, Martindale JL, Wang XT, Gorospe M, and Holbrook NJ. Effects of aging and calorie restriction of Fischer 344 rats on hepatocellular response to proliferative signals. Exp Gerontol 38: 431-439, 2003.
117. Jazwinski SM. Yeast longevity and aging-the mitochondrial connection. Mech Ageing Dev 126: 243-248, 2005.
118. Jones SJM, Riddle DL, Pouzyrev AT, Velculescu VE, Hillier LD, Eddy SR, Stricklin SL, Baillie DL, Waterston R, and Marra MA. Changes in gene expression associated with developmental arrest and longevity in Caenorhabditis elegans. Genome Res 11: 1346-1352, 2001.
119. Kacser A and Burns JA. Molecular democracy: who shares the controls? Biochem Soc Trans 7: 1149-1160, 1979.
120. Kanno T, Sato EE, Muranaka S, Fujita H, Fujiwara T, Utsumi T, Inoue M, and Utsumi K. Oxidative stress underlies the mechanism for Ca(2+)-induced permeability transition of mitochondria. Free Radic Res 38: 27-35, 2004.
121. Karanjawala ZE and Lieber MR. DNA damage and aging. Mech Ageing Dev 125: 405-416, 2004.
122. Kim HJ, Jung KJ, Yu BP, Cho CG, Choi JS, and Chung HY. Modulation of redox-sensitive transcription factors by calorie restriction during aging. Mech Ageing Dev 123: 1589-1595, 2002.
123. King MP and Attardi G. Isolation of human cell lines lacking mitochondrial DNA. Methods Enzymol 264: 304-313, 1996.
124. Kleist-Retzow J-C, Schauseil-Zipf U, Michalk DV, and Kunz WS. Mitochondrial diseases-an expanding spectrum of disorders and affected genes. Exp Physiol 88: 155-166, 2003.
125. Korshunov SS, Skulachev VP, and Starkov AA. High protonic potential actuates a mechanism of production of reactive oxygen species in mitochondria. FEBS Lett 416: 15-18, 1997.
126. Krause F, Reifschneider NH, Goto S, and Dencher NA. Active oligomeric ATP synthases in mammalian mitochondria. Biochem Biophys Res Commun 329: 583-590, 2005.
127. Kroger A and Klingenberg M. The kinetics of the redox reactions of ubiquinone related to the electron-transport activity in the respiratory chain. Eur J Biochem 34: 358-368, 1973.
128. Kudin AP, Bimpong-Buta NY-B, Vielhaber S, Elger CE, and Kunz WS. Characterization of superoxide-producing sites in isolated brain mitochondria. J Biol Chem 279: 4127-4135, 2004.
129. Kunz WS. Different metabolic properties of mitochondrial oxidative phosphorylation in different cell types-important implications for mitochondrial cytopathies. Exp Physiol 88: 140-154, 2003.
130. Kushnareva Y, Murphy AN, and Andreyev A. Complex I-mediated reactive oxygen species generation: modulation by cytochrome c and NAD(P)+ oxidation-reduction state. Biochem J 368: 545-553, 2002.
131. Laderman KA, Penny JR, Mazzucchelli F, Bresolin N, Scarlato G, and Attardi G. Aging-dependent functional alterations of mitochondrial DNA (mtDNA) from human fibroblasts transferred into mtDNA-less cells. J Biol Chem 271: 15891-15897, 1996.
132. Lavrovsky Y, Chatterjee B, Clark RA, and Roy AK. Role of redox-regulated transcription factors in inflammation, aging and age-related diseases. Exp Gerontol 35: 521-532, 2000.
133. Lee HC, Lu CY, Fahn HJ, and Wei YH. Aging- and smoking-associated alteration in the relative content of mitochondrial DNA in human lung. FEBS Lett 441: 292-296, 1998.
134. Lee HC and Wei YH. Mutation and oxidative damage of mitochondrial DNA and defective turnover of mitochondria in human aging. J Formos Med Assoc 96: 770-778, 1997.
135. Lee HC, Yin PH, Lu CY, Chi CW, and Wei YH. Increase of mitochondria and mitochondrial DNA in response to oxidative stress in human cells. Biochem J 348: 425-432, 2000.
136. Lee HC, Yin PH, Chi CW, and Wei YH. Increase in mitochondrial mass in human fibroblasts under oxidative stress and during replicative cell senescence. J Biomed Sci 9: 517-526, 2002.
137. Lenaz G. Role of mobility of redox components in the inner mitochondrial membrane. J Membr Biol 104: 193-209, 1988.
138. Lenaz G. Role of mitochondria in oxidative stress and ageing. Biochim Biophys Acta 1366: 53-67, 1998.
139. Lenaz G. Quinone specificity of complex I. Biochim Biophys Acta 1364: 207-221, 1998.
140. Lenaz G. A critical appraisal of the mitochondrial coenzyme Q pool. FEBS Lett 159: 151-155, 2001.
141. Lenaz G. The mitochondrial production of reactive oxygen species: mechanisms and implications in human pathology. IUBMB Life 52: 159-164, 2001.
142. Lenaz G, Baracca A, Carelli V, D'Aurelio M, Sgarbi G, and Solaini G. Bioenergetics of mitochondrial diseases associated with mtDNA mutations. Biochim Biophys Acta 1658: 89-94, 2004.
143. Lenaz G, Bovina C, D'Aurelio M, Fato R, Formiggini G, Genova ML, Giuliano G, Merlo Pich M, Paolucci U, Parenti Castelli G, and Ventura B. Role of mitochondria in oxidative stress and aging. Ann NY Acad Sci 959: 199-213, 2002.
144. Lenaz G, Bovina C, Formiggini G, and Parenti Castelli G. Mitochondria, oxidative stress, and antioxidant defences. Acta Biochim Pol 46: 1-21, 1999.
145. Lenaz G, D'Aurelio M, Merlo Pich M, Genova ML, Ventura B, Bovina C, Formiggini G, and Parenti Castelli G. Mitochondrial bioenergetics in aging. Biochim Biophys Acta 1459: 397-404, 2000.
146. Lenaz G, Fato R, Genova ML, Formiggini G, Parenti Castelli G, and Bovina C. Underevaluation of complex I activity by the direct assay of NADH-coenzyme Q reductase in rat liver mitochondria. FEBS Lett 366: 119-121, 1995.
147. Lezza AM, Pesce V, Cormio A, Fracasso F, Vecchiet J, Felzani G, Cantatore P, and Gadaleta MN. Increased expression of mitochondrial transcription factor A and nuclear respiratory factor-1 in skeletal muscle from aged human subjects. FEBS Lett 501: 74-78, 2001.
148. Li J and Holbrook NJ. Common mechanisms for declines in oxidative stress tolerance and proliferation with aging. Free Radic Biol Med 35: 292-299, 2003.
149. Li N, Ragheb K, Lawler G, Sturgis J, Rajwa B, Melendez JA, and Robinson JP. Mitochondrial complex I inhibitor rotenone induces apoptosis through enhancing mitochondrial reactive oxygen species production. J Biol Chem 278: 8516-8525, 2003.
150. Li Y and Trush MA. Diphenyleneiodonium, an NAD(P)H oxidase inhibitor, also potently inhibits mitochondrial reactive oxygen species production. Biochem Biophys Res Commun 253: 295-299, 1998.
151. Lincoln AJ, Donat N, Palmer G, and Prochaska LJ. Site-specific antibodies against hydrophilic domains of subunit III of bovine heart cytochrome c oxidase affect enzyme function. Arch Biochem Biophys 416: 81-91, 2003.
152. Linnane AW, Degli Esposti M, Generowicz M, Luff AR, and Nagley P. The universality of bioenergetic disease and amelioration with redox therapy. Biochim Biophys Acta 1271: 191-194, 1995.
153. Linnane AW, Marzuki S, Ozawa T, and Tanaka M. Mitochondrial DNA mutations as an important contributor to ageing and degenerative diseases. Lancet i, 642-645, 1989.
154. Liu Y, Fiskum G, and Schubert D. Generation of reactive oxygen species by the mitochondrial electron transport chain. J Neurochem 80: 780-787, 2002.
155. Liu VWS, Zhang C, Pang CY, Lee HC, Lu CY, Wei YH, and Nagley P. Independent occurrence of somatic mutations in mitochondrial DNA of human skin from subjects of various ages. Hum Mutat 11: 191-196, 1998.
156. Lopez-Lluch G, Barroso MP, Martin SF, Fernandez-Ayala DJM, Gomez-Diaz C, Villalba JM, and Navas P. Role of plasma membrane coenzyme Q on the regulation of apoptosis. Biofactors 9: 171-177, 1999.
157. Lopez-Torres M, Gredilla R, Sanz A, and Barja G. Influence of aging and long-term caloric restriction on oxygen radical generation and oxidative DNA damage in rat liver mitochondria. Free Radic Biol Med 32: 882-889, 2002.
158. MacAlpine DM, Kolesar J, Okamoto K, Butow RA, and Perlman PS. Replication and preferential inheritance of hypersuppressive petite mitochondrial DNA. EMBO J 20: 1807-1817, 2001.
159. Magnitsky S, Toulokhonova L, Yano T, Sled VD, Hagerhall C, Grivennikova V, Burbaev DS, Vinogradov AD, and Ohnishi T. EPR characterization of ubisemiquinones and iron-sulfur cluster N2: central components of the energy coupling in the NADH-ubiquinone oxidoreductase (complex I) in situ. J Bioenerg Biomembr 34: 193-208, 2002.
160. Majander A, Finel M, Savontaus M-L, Nikoskelainen E, and Wikstrom M. Catalytic activity of complex I in cell lines that possess replacement mutations in the ND genes in Leber's hereditary optic neuropathy. Eur J Biochem 239: 201-207, 1996.
161. Majander A, Huoponen K, Savontaus M-L, Nikoskelainen EK, and Wikstrom M. Electron transfer properties of NADH:ubiquinone reductase in the ND1/3460 and the ND4/11778 mutations of the Leber hereditary optic neuroretinopathy (LHON). FEBS Lett 292: 289-292, 1991.
162. Manczak M, Jung Y, Park BS, Partovi D, and Reddy PH. Time-course of mitochondrial gene expressions in mice brains: implications for mitochondrial dysfunction, oxidative damage, and cytochrome c in aging. J Neurochem 92: 494-504, 2005.
163. Mandavilli BS, Santos JH, and Van Houten B. Mitochondrial DNA repair and aging. Mutat Res 509: 127-151, 2002.
164. Manfredi G, Fu J, Ojaimi J, Sladock JE, Kwong JQ, Guy J, and Schon EA. Rescue of a deficiency in ATP synthesis by transfer of MTATP6, a mitochondrial DNA-encoded gene, to the nucleus. Nat Genet 30: 394-399, 2002.
165. Manfredi G, Schon EA, Moraes CT, Bonilla E, Berry GT, Sladky JT, and DiMauro S. A new mutation associated with MELAS is located in a mitochondrial DNA polypeptide-coding gene. Neuromuscul Disord 5: 391-398, 1995.
166. Mather MW and Rottenberg H. Intrinsic uncoupling of cytochrome c oxidase may cause the maternally inherited mitochondrial diseases MELAS and LHON. FEBS Lett 433: 93-97, 1998.
167. Matsuno-Yagi A and Yagi T. Introduction: complex I-an L-shaped black box. J Bioenerg Biomembr 33: 155-157, 2001.
168. Mattiazzi M, Vijayvergiya C, Gajewski CD, DeVivo DC, Lenaz G, Wiedmann M, and Manfredi G. The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants. Hum Mol Genet 13: 869-879, 2004.
169. McLennan H and Degli Esposti M. The contribution of mitochondrial respiratory complexes to the production of reactive oxygen species. J Bioenerg Biomembr 32: 153-162, 2000.
170. Melov S. Animal models of oxidative stress, aging, and therapeutic antioxidant interventions. Int J Biochem Cell Biol 34: 1395-1400, 2002.
171. Melov S, Coskun P, Patel M, Tuinstra M, Cottrell B, Jun AS, Zastawny TH, Dizdaroglu M, Goodman SI, Huang TT, Miziorko H, Epstein CJ, and Wallace DC. Mitochondrial disease in superoxide dismutase 2 mutant mice. Proc Natl Acad Sci USA 96: 846-851, 1999.
172. Merlo Pich M, Bovina C, Formiggini G, Cometti GG, Ghelli A, Parenti Castelli G, Genova ML, Marchetti M, Semeraro S, and Lenaz G. Inhibitor sensitivity of respiratory complex I in human platelets: a possible biomarker of ageing. FEBS Lett 380: 176-178, 1996.
173. Merlo Pich M, Raule N, Catani L, Fagioli ME, Faenza I, Cocco L, and Lenaz G. Increased transcription of mitochondrial genes for Complex I in human platelets during ageing. FEBS Lett 558: 19-22, 2004.
174. Meunier B. Site-directed mutations in the mitochondrially encoded subunits I and III of yeast cytochrome oxidase. Biochem J 354: 407-412, 2001
175. Michikawa Y, Mazzucchelli F, Bresolin N, Scarlato G, and Attardi G. Aging-dependent large accumulation of point mutations in the human mtDNA control region for replication. Science 286: 774-779, 1999.
176. Mills DA, Tan Z, Ferguson-Miller S, and Hosler. A role for subunit III in proton uptake into the D pathway and a possible proton exit pathway in Rhodobacter sphaeroides cytochrome c oxidase. Biochemistry 42: 7410-7417, 2003.
177. Miquel J, Economos AC, Fleming J, and Johnson JE Jr. Mitochondrial role in cell aging. Exp Gerontol 15: 575-591, 1980.
178. Mirò O, Casademont J, Casals E, Perea M, Urbano-Marquez A, Rustin P, and Cardellach F. Aging is associated with increased lipid peroxidation in human hearts, but not with mitochondrial respiratory chain enzyme defects. Cardiovasc Res 47: 624-631, 2000.
179. Miskin R, Masos T, Yahav S, Shinder D, and Globerson A. AlphaMUPA mice: a transgenic model for increased life span. Neurobiol Aging 20: 555-564, 1999.
180. Moraes CT. What regulates mitochondrial DNA copy number in animal cells? Trends Genet 17: 199-205, 2001.
181. Morgan-Hughes JA and Hanna MG. Mitochondrial encephalomyopathies: the enigma of genotype versus phenotype. Biochim Biophys Acta 1410: 125-145, 1999.
182. Morre' DM, Lenaz G, Morre' DJ. Surface oxidase and oxidative stress propagation in aging. J Exp Biol 203: 1513-1521, 2000.
183. Muller FL, Roberts AG, Bowman NK, and Kramer DM. Architecture of the Qo site of the cytochrome bcl complex probed by superoxide production. Biochemistry 42: 6493-6499, 2003.
184. Nagley P and Wei YH. Ageing and mammalian mitochondrial genetics. Trends Genet 14: 513-517, 1998.
185. Navarro F, Navas P, Burgess JR, Di Bello RJ, De Cabo R, Arroyo A, and Villalba JM. Vitamin E and selenium deficiency induces expression of the ubiquinone-dependent antioxidant system at the plasma membrane. FASEB J 12: 1665-1673, 1998.
186. Nijtmans LGJ, Henderson NS, Attardi G, and Holt LJ. Impaired ATP synthase assembly associated with a mutation in the human ATP synthase subunit 6 gene. J Biol Chem 276: 6755-6762, 2001.
187. Nohl H and Stolze K. Ubisemiquinones of the mitochondrial respiratory chain do not interact with molecular oxygen. Free Radic Res Commun 16: 409-419, 1992.
188. Okun JG, Lummen P, and Brandt U. Three classes of inhibitors share a common binding domain in mitochondrial complex I (NADH:ubiquinone oxidoreductase). J Biol Chem 274: 2625-2630, 1999.
189. Ohnishi T. Iron-sulfur clusters/semiquinones in complex I. Biochim Biophys Acta 1364: 186-206, 1998.
190. Ono T, Isobe K, Nakada K, and Hayashi JL. Human cells are protected from mitochondrial dysfunction by complementation of DNA products in fused mitochondria. Nat Genet 28: 272-275, 2001.
191. Osiewacz HD. Aging in fungi: role of mitochondria in Podospora anserina. Mech Ageing Dev 123: 755-764, 2002.
192. Osiewacz HD and Stumpferl S. Metabolism and aging in the filamentous fungus Podospora anserina. Arch Gerontol Geriatr 32: 185-197, 2001.
193. Ozawa T. Genetic and functional changes in mitochondria associated with aging. Physiol Rev 77: 425-464, 1997.
194. Pallotti F, Baracca A, Hernandez-Rosa E, Walzer WF, Solaini G, Lenaz G, Melzi D'Eril GV, Di Mauro S, Schon EA, and Davidson MM. Biochemical analysis of respiratory function in cybrid cell lines harbouring mitochondrial DNA mutations. Biochem J 384: 287-293, 2004.
195. Pamplona R, Barja G, and Portero-Otin M. Membrane fatty acid unsaturation, protection against oxidative stress, and maximum life span: a homeoviscous-longevity adaptation? Ann NY Acad Sci 959: 475-490, 2002.
196. Paradies G, Petrosillo G, Pistolese M, Di Venosa N, Federici A, and Ruggiero FM. Decrease in mitochondrial complex I activity in ischemic/reperfused rat heart: involvement of reactive oxygen species and cardiolipin. Circ Res 94: 53-59, 2004.
197. Parker WD, Boyson SJ, and Parks JK. Abnormalities of the electron transport chain in idiopathic Parkinson's disease. Ann Neurol 26: 719-723, 1989.
198. Pesce V, Cormio A, Fracasso F, Vecchiet J, Felzani G, Lezza AMS, Cantatore P, and Gadaleta MN. Age-related mitochondrial genotypic and phenotypic alterations in human skeletal muscle. Free Radic Biol Med 30: 1223-1233, 2001.
199. Petronilli V, Costantini P, Scorrano L, Colonna R, Passamonti S, and Bernardi P. The voltage sensor of the mitochondrial permeability transition pore is tuned by the oxidation-reduction state of vicinal thiols. Increase of the gating potential by oxidants and its reversal by reducing agents. J Biol Chem 269: 16638-16642, 1994.
200. Petrosillo G, Ruggiero FM, Di Venosa N, and Paradies G. Decreased complex III activity in mitochondria isolated from rat heart subjected to ischemia and reperfusion: role of reactive oxygen species and cardiolipin. FASEB J 17: 714-716, 2003.
201. Pfeiffer K, Gohil V, Stuart RA, Hunte C, Brandt U, Greenberg ML, and Schägger H. Cardiolipin stabilizes respiratory chain supercomplexes. J Biol Chem 278: 52873-52880, 2003.
202. Pitkänen S and Robinson BH. Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase. J Clin Invest 98: 345-351, 1996.
203. Polyak K, Li Y, Zhu H, Lengauer C, Willson JK, Markowitz SD, Trush MA, Kinzler KW, and Vogelstein B. Somatic mutations of the mitochondrial genome in human colorectal tumours. Nat Genet 20: 291-293, 1998.
204. Puigserver P and Spiegelmann BM. Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator. Endocr Rev 24: 78-90, 2003.
205. Richter C. Oxidative damage to mitochondrial DNA and its relationship to ageing. Int J Biochem Cell Biol 27: 647-653, 1995.
206. Richter C, Park JW, and Ames BN. Normal oxidative damage to mitochondrial and nuclear DNA is extensive. Proc Natl Acad Sci USA 85: 6465-6467, 1988.
207. Rose G, Passarino G, Carrieri G, Altomare K, Greco V, Bertolini S, Bonafè M, Franceschi C, and De Benedictis G. Paradoxes in longevity: sequence analysis of mtDNA haplogroup J in centenarians. Eur J Hum Genet 9: 701-707, 2001.
208. Rose G, Passarino G, Franceschi C, De and Benedictis G. The variability of the mitochondrial genome in human aging: a key for life and death? Int J Biochem Cell Biol 34: 1449-1460, 2002.
209. Rossignol R, Faustin B, Rocher C, Malgat M, Mazat JP, and Letellier T. Mitochondrial threshold effects. Biochem J 370: 751-762, 2003.
210. Rossignol R, Malgat M, Mazat JP, and Letellier T. Threshold effect and tissue specificity. Implication for mitochondrial cytopathies. J Biol Chem 274: 33426-33432, 1999.
211. Salvioli S, Storci G, Pinti M, Quaglino D, Moretti L, Merlo-Pich M, Lenaz G, Filosa S, Fico A, Bonafè M, Monti D, Troiano L, Nasi M, Cossarizza A, and Franceschi C. Apoptosis-resistant phenotype in HL-60-derived cells HCW-2 is related to changes in expression of stress-induced proteins that impact on redox status and mitochondrial metabolism. Cell Death Differ 10: 163-74, 2003.
212. Santorelli FM, Shanske S, Macaya A, DeVivo DC, and Di Mauro S. The mutation at nt 8993 of mitochondrial DNA is a common cause of Leigh's syndrome. Ann Neurol 34: 827-834, 1993.
213. Schägger H, De Coo R, Bauer MF, Hofmann S, Godinot C, and Brandt U. Significance of respirasomes for the assembly/stability of human respiratory chain complex I. J Biol Chem 279: 36349-36353, 2004.
214. Schägger H and Pfeiffer K. The ratio of oxidative phosphorylation complexes I-V in bovine heart mitochondria and the composition of respiratory chain supercomplexes. J Biol Chem 276: 37861-37867, 2001.
215. Schapira AHV. Mitochondrial dysfunction in neurodegenerative disorders. Biochim Biophys Acta 1366: 225-233, 1998.
216. Schon EA, Bonilla E, and Di Mauro S. Mitochondrial DNA mutations and pathogenesis. J Bioenerg Biomembr 29: 131-149, 1997.
217. Schon EA, Santa S, Pallotti F, and Girvin ME. Pathogenesis of primary defects in mitochondrial ATP synthesis. Cell Dev Biol 12: 441-448, 2001.
218. Schoneich C. Reactive oxygen species and biological aging: a mechanistic approach. Exp Gerontol 34: 19-34, 1999.
219. Schoneich C. Methionine oxidation by reactive oxygen species: reaction mechanisms and relevance to Alzheimer's disease. Biochim Biophys Acta 1703: 111-119, 2005.
220. Schultz BE and Chan SI. Structures and proton-pumping strategies of mitochondrial respiratory enzymes. Annu Rev Biophys Biomol Struct 30: 23-65, 2001.
221. Singh KK. Mitochondrial dysfunction is a common phenotype in aging and cancer. Ann NY Acad Sci 1019: 260-264, 2004.
222. Skulachev VP. Role of uncoupled and non-coupled oxidations in maintenance of safely low levels of oxygen and its one-electron reductants. Q Rev Biophys 29: 169-202, 1996.
223. Smit AJ and Lutgers HL. The clinical relevance of advanced glycation endproducts (AGE) and recent developments in pharmaceutics to reduce AGE accumulation. Curr Med Chem 11: 2767-2784, 2004.
224. Sohal RS, Mockett RJ, and Orr WC. Mechanisms of aging: an appraisal of the oxidative stress hypothesis. Free Radic Biol Med 33: 575-586, 2002.
225. Sohal RS and Sohal BH. Hydrogen peroxide release by mitochondria increases during aging. Mech Ageing Dev 57: 187-202, 1991.
226. Sohal RS, Sohal BH, and Orr WC. Mitochondrial superoxide and hydrogen peroxide generation, protein oxidative damage, and longevity in different species of flies. Free Radic Biol Med 19: 499-504, 1995.
227. Sohal RS, Svensson I, and Brunk UT. Hydrogen peroxide production by liver mitochondria in different species. Mech Ageing Dev 53: 209-215, 1990.
228. Sohal RS and Weindruch R. Oxidative stress, caloric restriction, and aging. Science 273: 59-63, 1996.
229. Speakman JR, Van Acker A, and Harper EJ. Age-related changes in the metabolism and body composition of three dog breeds and their relationship to life expectancy. Aging Cell 2: 265-275, 2003.
230. Speakman JR, Talbot DA, Selman C, Snart S, McLaren JS, Redman P, Krol E, Jackson DM, Johnson MS, and Brand MD. Uncoupled and surviving: individual mice with high metabolism have greater mitochondrial uncoupling and live longer. Aging Cell 3: 87-95, 2004.
231. Spencer CC, Howell CE, Wright AR, and Promislow DE. Testing an 'aging gene' in long-lived drosophila strains: increased longevity depends on sex and genetic background. Aging Cell 2: 123-130, 2003.
232. Spencer JP, Jenner P, Daniel SE, Lees AJ, Marsden DC, and Halliwell H. Conjugates of catecholamines with cysteine and GSH in Parkinson's disease: possible mechanisms of formation involving reactive oxygen species. J Neurochem 71: 2112-2122, 1998.
233. Stadtman ER. Protein oxidation and aging. Science 257: 1220-1224, 1992.
234. Staniek K and Nohl H. Are mitochondria a permanent source of reactive oxygen species? Biochim Biophys Acta 1460: 268-275, 2000.
235. +-translocating NADH:quinone oxidoreductase (NDH I) from Klebsiella pneumoniae and Escherichia coli: implications for the mechanism of redox-driven cation translocation by complex I. J Bioenerg Biomembr 33: 179-186, 2001.
236. Talbot DA, Lambert AJ, and Brand MD. Production of endogenous matrix superoxide from mitochondrial complex I leads to activation of uncoupling protein 3. FEBS Lett 556: 111-115, 2004.
237. Tanaka M, Gong J-S, Zhang J, Yamada Y, Borgeld H-J, and Yagi K. Mitochondrial genotype associated with longevity and its inhibitory effect on mutagenesis. Mech Ageing Dev 116: 65-76, 2000.
238. Tatuch Y, Christodoulou J, Feigenbaum A, Clarke JTR, Wherret J, Smith C, Rudd N, Pertrova-Benedict R, and Robinson BH. Heteroplasmic mtDNA mutation (T-G) at 8993 can cause Leigh disease when the percentage of abnormal mtDNA is high. Am J Hum Genet 50: 852-858, 1992.
239. Teismann P, Tieu K, Choi DK, Wu DC, Naini A, Hunot S, Vila M, Jackson-Lewis V, and Przedborski S. Cyclooxygenase-2 is instrumental in Parkinson's disease neurodegeneration. Proc Natl Acad Sci USA 100: 5473-5478, 2003.
240. Tiranti V, Corona P, Greco M, Taanman J-W, Carrara F, Lamantea E, Nijtmans L, Uziel G, and Zeviani M. A novel frameshift mutation of the mtDNA COIII gene leads to impaired assembly of cytochrome c oxidase in a patient affected by Leigh-like syndrome. Hum Mol Genet 18: 2733-2742, 2000.
241. Torroni A, Petrozzi M, D'Urbano L, Sellitto D, Zeviani M, Carrara F, Carducci C, Leuzi V, Barboni P, De Negri A, and Scozzari R. Haplotype and phylogenetic analyses suggest that one European-specific mtDNA background plays a role in the expression of Leber hereditary optic neuropathy by increasing the penetrance of the primary mutations 11778 and 14484. Am J Hum Genet 60: 1107-1121, 1997.
242. Trifunovic A, Wredenberg A, Falkenberg M, Spelbrink JN, Rovio AJ, Bruder CF, Bohlooly YM, Gedlof S, Oldfors A, Wibom R, Jacobs HT, and Larsson NG. Premature ageing in mice expressing defective mitochondrial DNA polymerase. Nature 429: 357-359, 2004.
243. Tsang WY and Lemire BD. The role of mitochondria in the life of the nematode, Caenorhabditis elegans. Biochim Biophys Acta 1638: 91-105, 2003.
244. Turrens, JF, Alexandre, A, and Lehninger AL. Ubisemiquinone is the electron donor for superoxide formation by complex III of heart mitochondria. Arch Biochem Biophys 237: 408-414, 1985.
245. Velculescu VE, Zhang L, Vogelstein B, and Kinzler KW. Serial analysis of gene expression. Science 270: 484-487, 1995.
246. Ventura B, Genova ML, Bovina C, Formiggini G, and Lenaz G. Control of oxidative phosphorylation by Complex I in rat liver mitochondria: implications for aging. Biochim Biophys Acta 1553: 249-260, 2002.
247. Von Zglinicki T, Burkle A, and Kirkwood TBL. Stress, DNA damage and ageing-an integrative approach. Exp Gerontol 36: 1049-1062, 2001.
248. Votyakova TV and Reynolds IJ. DeltaPsi(m)-dependent and -independent production of reactive oxygen species by rat brain mitochondria. J Neurochem 79: 266-277, 2001.
249. Vrablic AS, Albright CD, Craciunescu CN, Salganik RI, and Zeisel SH. Altered mitochondrial function and over-generation of reactive oxygen species precede the induction of apoptosis by 1-O-octadecyl-2-methyl-rac-glycero-3- phosphocholine in p53-defective hepatocytes. FASEB J 15: 1739-1744, 2001.
250. Wallace DC. Mitochondrial DNA sequence variation in human evolution and disease. Proc Natl Acad Sci USA 91: 8739-8746, 1994.
251. Wallace DC. Mouse models for mitochondrial disease. Am J Med Genet 106: 71-93, 2001.
252. Wallace DC. Animal models for mitochondrial disease. Methods Mol Biol 197: 3-54, 2002.
253. Wallace DC, Singh G, Lott MT, Hodge JA, Schurr TG, Lezza AM, Elsas LJ, and Nikoskelainen EK. Mitochondrial DNA mutation associated with Leber's hereditary optic neuropathy. Science 242: 1427-1430, 1988.
254. Wang Y, Michikawa Y, Mallidis C, Bai Y, Woodhouse L, Yarasheski KE, Miller CA, Askanas V, Engel WK, Bhasin S, and Attardi G. Muscle-specific mutations accumulate with aging in critical human mtDNA control sites for replication. Proc Natl Acad Sci USA 98: 4022-4027, 2001.
255. Wei YH and Lee HC. Oxidative stress, mitochondrial DNA mutation, and impairment of antioxidant enzymes in aging. Exp Biol Med 227: 671-682, 2002.
256. Yakes FM and Van Houten B. Mitochondrial DNA damage is more extensive and persists longer than nuclear DNA damage in human cells following oxidative stress. Proc Natl Acad Sci USA 94: 514-519, 1997.
257. Yan LJ and Sohal RS. Prevention of flight activity prolongs the life span of the housefly, Musca domestica, and attenuates the age-associated oxidative damage to specific mitochondrial proteins. Free Radic Biol Med 29: 1143-1150, 2000.
258. Yu BP. How diet influences the aging process of the rat. Proc Exp Biol Med 205: 97-105, 1994.
259. Zhang J, Asin-Cayuela J, Fish J, Michikawa Y, Bonafè M, Olivieri F, Passarino G, De Benedictis G, Franceschi C, and Attardi G. Strikingly higher frequency in centenarians and twins of mtDNA mutation causing remodeling of replication origin in leukocytes. Proc Natl Acad Sci USA 100: 1116-1121, 2003.
260. Zhang M, Mileykowskaya E, and Dowhan W. Gluing the respiratory chain together. Cardiolipin is required for supercomplex formation in the inner mitochondrial membrane. J Biol Chem 277: 43553-43556, 2002.
261. 2+. J Biol Chem 279: 4166-4174, 2004.
262. 2+. Relevance to Parkinson's disease. J Biol Chem 280: 15587-15594, 2005.