Mitochondrial respiratory chain dysfunction: Implications in neurodegeneration

Free Radical Biology and Medicine

Volumn 53, Issue 3, 2012, Pages 595-609

  Morán, María ^a,b   Moreno Lastres, David ^a,b   Marín Buera, Lorena ^a,b   Arenas, Joaquín ^a   Martín, Miguel A ^a,b   Ugalde, Cristina ^a,b  

^a HOSPITAL UNIVERSITARIO 12 DE OCTUBRE   (Spain)

^b CENTRO DE INVESTIGACIÓN BIOMÉDICA EN RED DE ENFERMEDADES RARAS CIBERER   (Spain)

Author keywords

Assembly; Free radicals; Mitochondria; Mitochondrial dynamics; Neurodegeneration; Reactive oxygen species; Respiratory chain dysfunction

Indexed keywords

ALPHA SYNUCLEIN; BETA SECRETASE; GAMMA SECRETASE; HOLOENZYME; HYDROGEN PEROXIDE; IRON SULFUR PROTEIN; LEUCINE RICH REPEAT KINASE 2; MITOFUSIN 2; REACTIVE OXYGEN METABOLITE; SUPEROXIDE DISMUTASE; UBIQUITIN;

ALZHEIMER DISEASE; BIOSYNTHESIS; CELL SURVIVAL; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENZYME ACTIVITY; FEEDBACK SYSTEM; GENE MUTATION; GENETIC POLYMORPHISM; HEREDITARY MOTOR SENSORY NEUROPATHY; HUMAN; HYPERGLYCEMIA; MITOCHONDRIAL RESPIRATION; NERVE DEGENERATION; NONHUMAN; OXIDATIVE PHOSPHORYLATION; PARKINSON DISEASE; PRIORITY JOURNAL; RESPIRATORY CHAIN; REVIEW; WOLF HIRSCHHORN SYNDROME;

ANIMALS; DNA, MITOCHONDRIAL; ELECTRON TRANSPORT; ELECTRON TRANSPORT CHAIN COMPLEX PROTEINS; HUMANS; MITOCHONDRIA; MITOCHONDRIAL DISEASES; MUTATION; NEURODEGENERATIVE DISEASES; OXIDATIVE PHOSPHORYLATION; OXIDATIVE STRESS;

EID: 84863515775     PISSN: 08915849     EISSN: 18734596     Source Type: Journal    
DOI: 10.1016/j.freeradbiomed.2012.05.009     Document Type: Review

References (256)

1. R. Acin-Perez, M.P. Bayona-Bafaluy, P. Fernandez-Silva, R. Moreno-Loshuertos, A. Perez-Martos, C. Bruno, C.T. Moraes, and J.A. Enriquez Respiratory complex III is required to maintain complex I in mammalian mitochondria Mol. Cell 13 2004 805 815 (Pubitemid 38438476)
2. R. Acin-Perez, P. Fernandez-Silva, M.L. Peleato, A. Perez-Martos, and J.A. Enriquez Respiratory active mitochondrial supercomplexes Mol. Cell 32 2008 529 539
3. M.K. Ahsan, I. Lekli, D. Ray, J. Yodoi, and D.K. Das Redox regulation of cell survival by the thioredoxin superfamily: an implication of redox gene therapy in the heart Antioxid. Redox Signaling 11 2009 2741 2758
4. M. Albrecht LRRK2 mutations and Parkinsonism Lancet 365 2005 1230
5. C. Alexander, M. Votruba, U.E. Pesch, D.L. Thiselton, S. Mayer, A. Moore, M. Rodriguez, U. Kellner, B. Leo-Kottler, G. Auburger, S.S. Battacharya, and B. Wissinger OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28 Nat. Genet. 26 2000 211 215
6. H.K. Anandatheerthavarada, G. Biswas, M.A. Robin, and N.G. Avadhani Mitochondrial targeting and a novel transmembrane arrest of Alzheimers amyloid precursor protein impairs mitochondrial function in neuronal cells J. Cell Biol. 161 2003 41 54 (Pubitemid 36459078)
7. E. Andres-Mateos, C. Perier, L. Zhang, B. Blanchard-Fillion, T.M. Greco, B. Thomas, H.S. Ko, M. Sasaki, H. Ischiropoulos, S. Przedborski, T.M. Dawson, and V.L. Dawson DJ-1 gene deletion reveals that DJ-1 is an atypical peroxiredoxin-like peroxidase Proc. Natl. Acad. Sci. USA 104 2007 14807 14812 (Pubitemid 350003224)
8. H. Antonicka, A. Mattman, C.G. Carlson, D.M. Glerum, K.C. Hoffbuhr, S.C. Leary, N.G. Kennaway, and E.A. Shoubridge Mutations in COX15 produce a defect in the mitochondrial heme biosynthetic pathway, causing early-onset fatal hypertrophic cardiomyopathy Am. J. Hum. Genet. 72 2003 101 114 (Pubitemid 36056846)
9. M.A. Aon, S. Cortassa, E. Marbán, and B. ORourke Synchronized whole cell oscillations in mitochondrial metabolism triggered by a local release of reactive oxygen species in cardiac myocytes J. Biol. Chem 278 2003 44735 44744 (Pubitemid 37377231)
10. C.R. Arthur, S.L. Morton, L.D. Dunham, P.M. Keeney, and J.P. Bennett Jr. Parkinsons disease brain mitochondria have impaired respirasome assembly, age-related increases in distribution of oxidative damage to mtDNA and no differences in heteroplasmic mtDNA mutation abundance Mol. Neurodegener 4 2009 37
11. H. Atamna Heme binding to amyloid-beta peptide: mechanistic role in Alzheimers disease J. Alzheimers Dis 10 2006 255 266 (Pubitemid 44763996)
12. H. Atamna, and W.H. Frey 2nd A role for heme in Alzheimers disease: heme binds amyloid beta and has altered metabolism Proc. Natl. Acad. Sci. USA 101 2004 11153 11158 (Pubitemid 38989599)
13. H. Atamna, and K. Boyle Amyloid-beta peptide binds with heme to form a peroxidase: relationship to the cytopathologies of Alzheimers disease Proc. Natl. Acad. Sci. USA 103 2006 3381 3386 (Pubitemid 43346478)
14. D. Bach, S. Pich, F.X. Soriano, N. Vega, B. Baumgartner, J. Oriola, J.R. Daugaard, J. Lloberas, M. Camps, J.R. Zierath, R. Rabasa-Lhoret, H. Wallberg-Henriksson, M. Laville, M. Palacín, H. Vidal, F. Rivera, M. Brand, and A. Zorzano Mitofusin-2 determines mitochondrial network architecture and mitochondrial metabolism: a novel regulatory mechanism altered in obesity J. Biol. Chem. 278 2003 17190 17197 (Pubitemid 36799599)
15. D. Bach, D. Naon, S. Pich, F.X. Soriano, N. Vega, J. Rieusset, M. Laville, C. Guillet, Y. Boirie, H. Wallberg-Henriksson, M. Manco, M. Calvani, M. Castagneto, M. Palacín, G. Mingrone, J.R. Zierath, H. Vidal, and A. Zorzano Expression of Mfn2, the Charcot-Marie-Tooth neuropathy type 2 A gene, in human skeletal muscle: effects of type 2 diabetes, obesity, weight loss, and the regulatory role of tumor necrosis factor alpha and interleukin-6 Diabetes 54 2005 2685 2693 (Pubitemid 41233591)
16. K. Banerjee, M. Sinha, L. Pham Cle, S. Jana, D. Chanda, R. Cappai, and S. Chakrabarti Alpha-synuclein induced membrane depolarization and loss of phosphorylation capacity of isolated rat brain mitochondria: implications in Parkinsons disease FEBS Lett 584 2010 1571 1576
17. M.J. Barsoum, H. Yuan, A.A. Gerencser, G. Liot, Y. Kushnareva, S. Gräber, I. Kovacs, W.D. Lee, J. Waggoner, J. Cui, A.D. White, B. Bossy, J.C. Martinou, R.J. Youle, S.A. Lipton, M.H. Ellisman, G.A. Perkins, and E. Bossy-Wetzel Nitric oxide-induced mitochondrial fission is regulated by dynamin-related GTPases in neurons EMBO J 25 2006 3900 3911 (Pubitemid 44300275)
18. R.V. Baxter, K. Ben Othmane, J.M. Rochelle, J.E. Stajich, C. Hulette, S. Dew-Knight, F. Hentati, M. Ben Hamida, S. Bel, J.E. Stenger, J.R. Gilbert, M.A. Pericak-Vance, and J.M. Vance Ganglioside-induced differentiation-associated protein-1 is mutant in Charcot-Marie-Tooth disease type 4 A/8q21 Nat. Genet. 30 2002 21 22
19. A.C. Bayne, R.J. Mockett, W.C. Orr, and R.S. Sohal Enhanced catabolism of mitochondrial superoxide/hydrogen peroxide and aging in transgenic Drosophila Biochem. J. 391 2005 277 284 (Pubitemid 41532424)
20. G. Benard, N. Bellance, D. James, P. Parrone, H. Fernandez, T. Letellier, and R. Rossignol Mitochondrial bioenergetics and structural network organization J. Cell Sci. 120 2007 838 848 (Pubitemid 46523485)
21. A. Bender, K.J. Krishnan, C.M. Morris, G.A. Taylor, A.K. Reeve, R.H. Perry, E. Jaros, J.S. Hersheson, J. Betts, T. Klopstock, R.W. Taylor, and D.M. Turnbull High levels of mitochondrial DNA deletions in substantia nigra neurons in aging and Parkinson disease Nat. Genet. 38 2006 515 517
22. S. Beretta, L. Mattavelli, G. Sala, L. Tremolizzo, A.H. Schapira, A. Martinuzzi, V. Carelli, and C. Ferrarese Leber hereditary optic neuropathy mtDNA mutations disrupt glutamate transport in cybrid cell lines Brain 127 2004 2183 2192 (Pubitemid 39382227)
23. S. Beretta, J.P. Wood, B. Derham, G. Sala, L. Tremolizzo, C. Ferrarese, and N.N. Osborne Partial mitochondrial complex I inhibition induces oxidative damage and perturbs glutamate transport in primary retinal cultures: relevance to Leber hereditary optic neuropathy (LHON) Neurobiol. Dis. 24 2006 308 317 (Pubitemid 44572749)
24. C. Bianchi, M.L. Genova, G. Parenti Castelli, and G. Lenaz The mitochondrial respiratory chain is partially organized in a supercomplex assembly: kinetic evidence using flux control analysis J. Biol. Chem. 279 2004 36562 36569 (Pubitemid 39128999)
25. E.J. Boekema, and H.P. Braun Supramolecular structure of the mitochondrial oxidative phosphorylation system J. Biol. Chem. 282 2007 1 4 (Pubitemid 47076588)
26. M.D. Brown, F. Sun, and D.C. Wallace Clustering of Caucasian Leber hereditary optic neuropathy patients containing the 11778 or 14484 mutations on an mtDNA lineage Am. J. Hum. Genet. 60 1997 381 387 (Pubitemid 27058409)
27. M.D. Brown, E. Starikovskaya, O. Derbeneva, S. Hosseini, J.C. Allen, I.E. Mikhailovskaya, R.I. Sukernik, and D.C. Wallace The role of mtDNA background in disease expression: a new primary LHON mutation associated with Western Eurasian haplogroup J. Hum. Genet. 110 2002 130 138 (Pubitemid 36067429)
28. S.M. Budde, L.P. van den Heuvel, A.J. Janssen, R.J. Smeets, C.A. Buskens, L. DeMeirleir, R. Van Coster, M. Baethmann, T. Voit, J.M. Trijbels, and J.A. Smeitink Combined enzymatic complex I and III deficiency associated with mutations in the nuclear encoded NDUFS4 gene Biochem. Biophys. Res. Commun. 275 2000 63 68 (Pubitemid 30663630)
29. M.J. Calkins, M. Manczak, P. Mao, U. Shirendeb, and P.H. Reddy Impaired mitochondrial biogenesis, defective axonal transport of mitochondria, abnormal mitochondrial dynamics and synaptic degeneration in a mouse model of Alzheimers disease Hum. Mol. Genet. 20 2011 4515 4529
30. M.A. Calvaruso, P. Willems, M. van den Brand, F. Valsecchi, S. Kruse, R. Palmiter, J. Smeitink, and L. Nijtmans Mitochondrial complex III stabilizes complex I in the absence of NDUFS4 to provide partial activity Hum. Mol. Genet. 21 2011 115 120
31. L. Canevari, J.B. Clark, and T.E. Bates Beta-Amyloid fragment 25-35 selectively decreases complex IV activity in isolated mitochondria FEBS Lett 457 1999 131 134 (Pubitemid 29387424)
32. V. Carelli, A. Ghelli, M. Ratta, E. Bacchilega, S. Sangiorgi, R. Mancini, V. Leuzzi, P. Cortelli, P. Montagna, E. Lugaresi, and M. Degli Esposti Lebers hereditary optic neuropathy: biochemical effect of 11778/ND4 and 3460/ND1 mutations and correlation with the mitochondrial genotype Neurology 48 1997 1623 1632
33. V. Carelli, A. Achilli, M.L. Valentino, C. Rengo, O. Semino, M. Pala, A. Olivieri, M. Mattiazzi, F. Pallotti, F. Carrara, M. Zeviani, V. Leuzzi, C. Carducci, B. Simionati, L. Mednieta, S. Salomao, R. Belfort Jr., A.A. Sadun, and A. Torroni Haplogroup effects and recombination of mitochondrial DNA: novel clues from the analysis of Leber hereditary optic neuropathy pedigrees Am. J. Hum. Genet. 78 2006 564 574
34. V. Carelli, C. La Morgia, M.L. Valentino, P. Barboni, F.N. Ross-Cisneros, and A.A. Sadun Retinal ganglion cell neurodegeneration in mitochondrial inherited disorders Biochim. Biophys. Acta 1787 2009 518 528
35. C.S. Casley, L. Canevari, J.M. Land, J.B. Clark, and M.A. Sharpe Beta-amyloid inhibits integrated mitochondrial respiration and key enzyme activities J. Neurochem. 80 2002 91 100 (Pubitemid 34614581)
36. C. Caspersen, N. Wang, J. Yao, A. Sosunov, X. Chen, J.W. Lustbader, H.W. Xu, D. Stern, G. McKhann, and S.D. Yan Mitochondrial Abeta: a potential focal point for neuronal metabolic dysfunction in Alzheimers disease FASEB J 19 2005 2040 2041 (Pubitemid 41759758)
37. R. Castellani, K. Hirai, G. Aliev, K.L. Drew, A. Nunomura, A. Takeda, A.D. Cash, M.E. Obrenovich, G. Perry, and M.A. Smith Role of mitochondrial dysfunction in Alzheimers disease J. Neurosci. Res. 70 2002 357 360 (Pubitemid 35222373)
38. H. Chen, and D.C. Chan Mitochondrial dynamics - fusion, fission, movement, and mitophagy in neurodegenerative diseases Hum. Mol. Genet. 18 2009 R169 176
39. S.J. Chinta, J.K. Mallajosyula, A. Rane, and J.K. Andersen Mitochondrial alpha-synuclein accumulation impairs complex I function in dopaminergic neurons and results in increased mitophagy in vivo Neurosci. Lett. 486 2010 235 239
40. S.J. Chinta, and J.K. Andersen Nitrosylation and nitration of mitochondrial complex I in Parkinsons disease Free Radic. Res. 45 2011 53 58
41. D.H. Cho, T. Nakamura, J. Fang, P. Cieplak, A. Godzik, Z. Gu, and S. Lipton S-Nitrosylation of Drp1 mediates β-amyloid-related mitochondrial fission and neuronal injury Science 324 2009 102 105
42. M.R. Cookson The role of leucine-rich repeat kinase 2 (LRRK2) in Parkinsons disease Nat. Rev. Neurosci. 11 2010 791 796
43. C.M. Cruciat, K. Hell, H. Folsch, W. Neupert, and R.A. Stuart Bcs1p, an AAA-family member, is a chaperone for the assembly of the cytochrome bc(1) complex EMBO J 18 1999 5226 5233 (Pubitemid 29465573)
44. A. Cuesta, L. Pedrola, T. Sevilla, J. Garcia-Planells, M.J. Chumillas, F. Mayordomo, E. LeGuern, I. Marin, J.J. Vilchez, and F. Palau The gene encoding ganglioside-induced differentiation-associated protein 1 is mutated in axonal Charcot-Marie-Tooth type 4 A disease Nat. Genet. 30 2002 22 25
45. R.K. Dagda, S.J. Cherra 3rd, S.M. Kulich, A. Tandon, D. Park, and C.T. Chu Loss of PINK1 function promotes mitophagy through effects on oxidative stress and mitochondrial fission J. Biol. Chem. 284 2009 13843 13855
46. A. Daiber Redox signaling (cross-talk) from and to mitochondria involves mitochondrial pores and reactive oxygen species Biochim. Biophys. Acta 1797 2010 897 906
47. S.R. Danielson, J.M. Held, M. Oo, R. Riley, B.W. Gibson, and J.K. Andersen Quantitative mapping of reversible mitochondrial complex I cysteine oxidation in a Parkinson disease mouse model J. Biol. Chem. 286 2011 7601 7608
48. M. DAurelio, C.D. Gajewski, G. Lenaz, and G. Manfredi Respiratory chain supercomplexes set the threshold for respiration defects in human mtDNA mutant cybrids Hum. Mol. Genet. 15 2006 2157 2169 (Pubitemid 43923405)
49. M. DAurelio, C. Vives-Bauza, M.M. Davidson, and G. Manfredi Mitochondrial DNA background modifies the bioenergetics of NARP/MILS ATP6 mutant cells Hum. Mol. Genet. 19 2010 374 386
50. D.C. David, S. Hauptmann, I. Scherping, K. Schuessel, U. Keil, P. Rizzu, R. Ravid, S. Drose, U. Brandt, W.E. Muller, A. Eckert, and J. Gotz Proteomic and functional analyses reveal a mitochondrial dysfunction in P30L Tau transgenic mice J. Biol. Chem. 25 2005 23802 23814 (Pubitemid 40884866)
51. C. Delettre, G. Lenaers, J.M. Griffoin, N. Gigarel, C. Lorenzo, P. Belenguer, L. Pelloquin, J. Grosgeorge, C. Turc-Carel, E. Perret, C. Astaire-Dequeter, L. Las Quellec, B. Arnaud, B. Ducommun, J. Kaplan, and C.P. Hamel Nuclear gene OPA1, encoding a mitochondrial dynamin-related protein, is mutated in dominant optic atrophy Nat. Genet. 26 2000 207 210
52. S.A. Detmer, and D.C. Chan Functions and dysfunctions of mitochondrial dynamics Nat. Rev 8 2007 870 879 (Pubitemid 47622562)
53. F. Devaux, G. Lelandais, M. Garcia, S. Goussard, and C. Jacq Posttranscriptional control of mitochondrial biogenesis: spatio-temporal regulation of the protein import process FEBS Lett 584 2010 4273 4279
54. L. Devi, B.M. Prabhu, D.F. Galati, N.G. Avadhani, and H.K. Anandatheerthavarada Accumulation of amyloid precursor protein in the mitochondrial import channels of human Alzheimers disease brain is associated with mitochondrial dysfunction J. Neurosci. 26 2006 9057 9068 (Pubitemid 44319397)
55. L. Devi, V. Raghavendran, B.M. Prabhu, N.G. Avadhani, and H.K. Anandatheerthavarada Mitochondrial import and accumulation of alpha-synuclein impair complex I in human dopaminergic neuronal cultures and Parkinson disease brain J. Biol. Chem. 283 2008 9089 9100
56. F. Diaz, H. Fukui, S. Garcia, and C.T. Moraes Cytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblasts Mol. Cell. Biol. 26 2006 4872 4881 (Pubitemid 43955805)
57. F. Diaz, J.A. Enriquez, and C.T. Moraes Cells lacking Rieske iron sulfur protein have a ROS-associated decrease in respiratory complexes I and IV Mol. Cell Biol. 32 2011 415 429
58. S. DiMauro, and M. Hirano Pathogenesis and treatment of mitochondrial disorders Adv. Exp. Med. Biol. 652 2009 139 170
59. 2-stimulated ATP production in human complex I deficiency J. Mol. Med. (Berlin) 87 2009 515 522
60. M.W. Dodson, and M. Guo Pink1, Parkin, DJ-1 mitochondrial dysfunction in Parkinsons disease Curr. Opin. Neurobiol. 17 2007 331 337 (Pubitemid 46880329)
61. H. Du, and S.S. Yan Mitochondrial medicine for neurodegenerative diseases Int. J. Biochem. Cell Biol. 42 2010 560 572
62. N.V. Dudkina, H. Eubel, W. Keegstra, E.J. Boekema, and H.P. Braun Structure of a mitochondrial supercomplex formed by respiratory-chain complexes I and III Proc. Natl. Acad. Sci. USA 102 2005 3225 3229 (Pubitemid 40328027)
63. N.V. Dudkina, M. Kudryashev, H. Stahlberg, and E.J. Boekema Interaction of complexes I, III, and IV within the bovine respirasome by single particle cryoelectron tomography Proc. Natl. Acad. Sci. USA 108 2011 15196 15200
64. S. Endele, M. Fuhry, S.J. Pak, B.U. Zabel, and A. Winterpacht LETM1, a novel gene encoding a putative EF-hand Ca(2)-binding protein, flanks the Wolf-Hirschhorn syndrome (WHS) critical region and is deleted in most WHS patients Genomics 60 1999 218 225 (Pubitemid 29463680)
65. A.R. Esteves, D.M. Arduino, R.H. Swerdlow, C.R. Oliveira, and S.M. Cardoso Oxidative stress involvement in alpha-synuclein oligomerization in Parkinsons disease cybrids Antioxid. Redox Signaling 11 2009 439 448
66. N. Exner, B. Treske, D. Paquet, K. Holmstrom, C. Schiesling, S. Gispert, I. Carballo-Carbajal, D. Berg, H.H. Hoepken, T. Gasser, R. Kruger, K.F. Winklhofer, F. Vogel, A.S. Reichert, G. Auburger, P.J. Kahle, B Schmidt, and C. Haaas Loss-of-function of human PINK1 results in mitochondrial pathology and can be rescued by parkin J. Neurosci. 27 2007 12413 12418 (Pubitemid 350085485)
67. J.C. Fernandez-Checa, A. Fernandez, A. Morales, M. Mari, C. Garcia-Ruiz, and A. Colell Oxidative stress and altered mitochondrial function in neurodegenerative diseases: lessons from mouse models CNS Neurol. Disord. Drug Targets 9 2010 439 454
68. D. Fernandez-Moreira, C. Ugalde, R. Smeets, R.J. Rodenburg, E. Lopez-Laso, M.L. Ruiz-Falco, P. Briones, M.A. Martin, J.A. Smeitink, and J. Arenas X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy Ann. Neurol. 61 2007 73 83 (Pubitemid 46214246)
69. E. Fernandez-Vizarra, M. Bugiani, P. Goffrini, F. Carrara, L. Farina, E. Procopio, A. Donati, G. Uziel, I. Ferrero, and M. Zeviani Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy Hum. Mol. Genet. 16 2007 1241 1252 (Pubitemid 47062720)
70. E. Fernandez-Vizarra, V. Tiranti, and M. Zeviani Assembly of the oxidative phosphorylation system in humans: what we have learned by studying its defects Biochim. Biophys. Acta 1793 2009 200 211
71. M. Floreani, E. Napoli, A. Martinuzzi, G. Pantano, V. De Riva, R. Trevisan, E. Bisetto, L. Valente, V. Carelli, and F. Dabbeni-Sala Antioxidant defences in cybrids harboring mtDNA mutations associated with Lebers hereditary optic neuropathy FEBS J 272 2005 1124 1135 (Pubitemid 40314932)
72. F. Fontanesi, I.C. Soto, and A. Barrientos Cytochrome c oxidase biogenesis: new levels of regulation IUBMB Life 60 2008 557 568
73. I. Fridovich Mitochondria: are they the seat of senescence? Aging Cell 3 2004 13 16 (Pubitemid 40246656)
74. H. Fukui, and C.T. Moraes The mitochondrial impairment, oxidative stress and neurodegeneration connection: reality or just an attractive hypothesis? Trends Neurosci 31 2008 251 256
75. M.E. Gegg, J.M. Cooper, K.Y. Chau, M. Rojo, A.H. Schapira, and J.W. Taanman Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin- dependent manner upon induction of mitophagy Hum. Mol. Genet. 19 2010 4861 4870
76. S. Geisler, K.M. Holmstrom, A. Treis, D. Skujat, S.S. Weber, F.C. Fiesel, P.J. Kahle, and W. Springer The PINK1/Parkin-mediated mitophagy is compromised by PD-associated mutations Autophagy 6 2010 871 878
77. V. Geromel, N. Kadhom, I. Cebalos-Picot, O. Ouari, A. Polidori, A. Munnich, A. Rotig, and P. Rustin Superoxide-induced massive apoptosis in cultured skin fibroblasts harboring the neurogenic ataxia retinitis pigmentosa (NARP) mutation in the ATPase-6 gene of the mitochondrial DNA Hum. Mol. Genet. 10 2001 1221 1228 (Pubitemid 32487545)
78. D. Ghezzi, P. Arzuffi, and M. Zordan Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies Nat. Genet 43 2011 259 263
79. M.C. Gil Borlado, D. Moreno Lastres, M. Gonzalez Hoyuela, M. Morán, A. Blazquez, R. Pello, L. Marin Buera, T. Gabaldon, J.J. Garcia Penas, M.A. Martin, J. Arenas, and C. Ugalde Impact of the mitochondrial genetic background in complex III deficiency PLoS One 5 2010 e12801
80. E. Gomez-Tortosa, K. Newell, M.C. Irizarry, M. Albert, J.H. Growdon, and B.T. Hyman Clinical and quantitative pathologic correlates of dementia with Lewy bodies Neurology 53 1999 1284 1291 (Pubitemid 29480089)
81. R. Gonzalo, E. Garcia-Arumi, D. Llige, R. Marti, A. Solano, J. Montoya, J. Arenas, and A.L. Andreu Free radicals-mediated damage in transmitochondrial cells harboring the T14487C mutation in the ND6 gene of mtDNA FEBS Lett 579 2005 6909 6913 (Pubitemid 41773708)
82. L. Gros, M.K. Saparbaev, and J. Laval Enzymology of the repair of free radicals-induced DNA damage Oncogene 21 2002 8905 8925 (Pubitemid 36110821)
83. O. Guillery, F. Malka, P. Frachon, D. Milea, M. Rojo, and A. Lombes Modulation of mitochondrial morphology by bioenergetics defects in primary human fibroblasts Neuromuscul. Disord 18 2008 319 330
84. R.D. Guzy, B. Sharma, E. Bell, N.D. Chandel, and P.T. Schumacker Loss of the SdhB, but not the SdhA, subunit of complex II triggers reactive oxygen species-dependent hypoxia-inducible factor activation and tumorigenesis Mol. Cell. Biol. 28 2008 718 731
85. B.J. Hanson, R.A. Capaldi, M.F. Marusich, and S.W. Sherwood An immunocytochemical approach to detection of mitochondrial disorders J. Histochem. Cytochem. 50 2002 1281 1288 (Pubitemid 35191472)
86. C.A. Hansson Petersen, N. Alikhani, H. Behbahani, B. Wiehager, P.F. Pavlov, I. Alafuzoff, V. Leinonen, A. Ito, B. Winblad, E. Glaser, and M. Ankarcrona The amyloid beta-peptide is imported into mitochondria via the TOM import machinery and localized to mitochondrial cristae Proc. Natl. Acad. Sci. USA 105 2008 13145 13150
87. Z. Harder, R. Zunino, and H. McBride Sumo1 conjugates mitochondrial substrates and participates in mitochondrial fission Curr. Biol. 14 2004 340 345 (Pubitemid 38300214)
88. D. Harman Aging: a theory based on free radical and radiation chemistry J. Gerontol. 11 1956 298 300
89. C. Henchcliffe, and M.F. Beal Mitochondrial biology and oxidative stress in Parkinson disease pathogenesis Nat. Clin. Pract. Neurol. 4 2008 600 609
90. J.T. Hinson, V.R. Fantin, J. Schonberger, N. Breivik, G. Siem, B. McDonough, P. Sharma, I. Keogh, R. Godinho, F. Santos, A. Esparza, Y. Nicolau, E. Selvaag, B.H. Cohen, C.L. Hoppel., L. Tranebjaerg, R.D. Eavey, and C.E. Seidman Missense mutations in the BCS1L gene as a cause of the Bjornstad syndrome New Engl. J. Med 356 2007 809 819 (Pubitemid 46294613)
91. K. Hirai, G. Aliev, A. Nunomura, H. Fujioka, R.L. Russell, C.S. Atwood, A.B. Johnson, Y. Kress, H.V. Vinters, M. Tabaton, S. Shimohama, A.D. Cash, S.L. Siedlak, P.L. Harris, P.K. Jones, R.B. Petersen, G. Perry, and M.A. Smith Mitochondrial abnormalities in Alzheimers disease J. Neurosci. 21 2001 3017 3023
92. S. Hofmann, M. Jaksch, R. Bezold, S. Mertens, S. Aholt, A. Paprotta, and K.D. Gerbitz Population genetics and disease susceptibility: characterization of central European haplogroups by mtDNA gene mutations, correlation with D loop variants and association with disease Hum. Mol. Genet. 6 1997 1835 1846 (Pubitemid 27460358)
93. J. Holzmann, P. Frank, E. Löffler, K.L. Bennett, C. Gerner, and W. Rossmanith RNase P without RNA: identification and functional reconstitution of the human mitochondrial tRNA processing enzyme Cell 135 2008 462 474
94. W.K. Hong, E.H. Han, D.G. Kim, J.Y. Ahn, J.S. Park, and B.G. Han Amyloid-beta-peptide reduces the expression level of mitochondrial cytochrome oxidase subunits Neurochem. Res. 32 2007 1483 1488 (Pubitemid 47094410)
95. H.T. Hornig-Do, T. Tatsuta, A. Buckermann, M. Bust, G. Kollberg, A. Rötig, M. Hellmich, L. Nijtmans, and R.J. Wiesner Nonsense mutations in the COX1 subunit impair the stability of respiratory chain complexes rather than their assembly EMBO J 31 2012 1293 1307
96. G. Hudson, V. Carelli, L. Spruijt, M. Gerards, C. Mowbray, A. Achilli, A. Pyle, J. Elson, N. Howell, C. La Morgia, M.L. Valentino, K. Huoponen, M.L. Savontaus., E. Nikoskeilainen, A.A. Sadun, S.R. Salomao, R. Belfort Jr., P. Griffiths, P.Y. Man., R.F. de Coo., R. Horvath., M. Zeviani, H.J Smeets, A. Torroni, and P.F. Chinnery Clinical expression of Leber hereditary optic neuropathy is affected by the mitochondrial DNA-haplogroup background Am. J. Hum. Genet. 81 2007 228 233 (Pubitemid 47236071)
97. B.G. Hughes, and S. Hekimi A mild impairment of mitochondrial electron transport has sex-specific effects on lifespan and aging in mice PLoS One 6 2011 e26116
98. E.C. Ienco, C. Simoncini, D. Orsucci, L. Petrucci, M. Filosto, M. Mancuso, and G. Siciliano May "mitochondrial Eve" and mitochondrial haplogroups play a role in neurodegeneration and Alzheimers disease? Int J. Alzheimers Dis. 2011 2011 709061
99. I. Irrcher, H. Aleyasin, E.L. Seifert, S. Chhabra, M. Philips, A.K. Lutz, M.W. Rousseaux, L. Bevilacqua, A. Jahani-Asl, S. Callaghan, J.G. MacLaurin, K.F. Winklhofer, P. Rizzu, P. Rippstein, R.H. Kim, C.X. Chen, E.A. Fon, R.S. Slack, M.E. Harper, H.M. McBride, T.W. Mak, and D.S. Park Loss of the Parkinsons disease-linked gene DJ-1 perturbs mitochondrial dynamics Hum. Mol. Genet. 19 2010 3734 3746
100. L.M. Ittner, and J. Gotz Amyloid-β and tau - a toxic pas de deux in Alzheimers disease Nat. Rev 12 2011 67 72
101. A. Iuso, S. Scacco, C. Piccoli, F. Bellomo, V. Petruzzella, R. Trentadue, M. Minuto, M. Ripoli, N. Capitanio, M. Zeviani, and S. Papa. Dysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex I J. Biol. Chem. 281 2006 10374 10380 (Pubitemid 43864576)
102. A. Jahani-Asl, E.C. Cheung, M. Neuspiel, J.G. MacLaurin, A. Fortin, D.S. Park, H.M. McBride, and R.S. Slack Mitofusin 2 protects cerebellar granule neurons against injury-induced cell death J. Biol. Chem. 282 2007 23788 23798 (Pubitemid 47328040)
103. A. Jahani-Asl, K. Pilon-Larose, W. Xu, J.G. MacLaurin, D.S. Park, H.M. McBride, and R.S. Slack The mitochondrial inner membrane GTPase, optic atrophy 1 (Opa1), restores mitochondrial morphology and promotes neuronal survival following excitotoxicity J. Biol. Chem. 286 2011 4772 4782
104. M. Jendrach, S. Mai, S. Pohl, M. Vöth, and J. Bereiter-Hahn Short- and long-term alterations of mitochondrial morphology, dynamics and mtDNA after transient oxidative stress Mitochondrion 8 2008 293 304
105. P. Jenner Altered mitochondrial function, iron metabolism and glutathione levels in Parkinsons disease Acta Neurol. Scand Suppl. 146 1993 3 13
106. B. Kadenbach, R. Ramzan, and S. Vogt Degenerative diseases, oxidative stress and cytochrome c oxidase function Trends Mol. Med. 15 2009 139 147
107. F. Kamp, N. Exner, A.K. Lutz, N. Wender, J. Hegermann, B. Brunner, B. Nuscher, T. Bartels, A. Giese, K. Beyer, S. Eimer, K.F. Winklhofer, and C. Haaas Inhibition of mitochondrial fusion by alpha-synuclein is rescued by PINK1, Parkin and DJ-1 EMBO J 29 2010 3571 3589
108. S.S. Karuppagounder, H. Xu, Q. Shi, L.H. Chen, S. Pedrini, D. Pechman, H. Baker, M.F. Beal, S.E. Gandy, and G.E. Gibson Thiamine deficiency induces oxidative stress and exacerbates the plaque pathology in Alzheimers mouse model Neurobiol. Aging 30 2009 1587 1600
109. S. Kawajiri, S. Saiki, S. Sato, F. Sato, T. Hatano, H. Eguchi, and N. Hattori PINK1 is recruited to mitochondria with parkin and associates with LC3 in mitophagy FEBS Lett 584 2010 1073 1079
110. P.J. Khandelwal, S.B. Dumanis, L.R. Feng, K. Maguire-Zeiss, G. Rebeck, H.A. Lashuel, and C.E. Moussa Parkinson-related parkin reduces α-synuclein phosphorylation in a gene transfer model Mol. Neurodegener 5 2010 47
111. I. Kim, S. Rodriguez-Enriquez, and J.J. Lemasters Selective degradation of mitochondria by mitophagy Arch. Biochem. Biophys. 462 2007 245 253 (Pubitemid 46876640)
112. Y. Kim, J. Park, S. Kim, S. Song, S.K. Kwon, S.H. Lee, T. Kitada, J.M. Kim, and J. Chung PINK1 controls mitochondrial localization of parkin through direct phosphorylation Biochem. Biophys. Res. Commun. 377 2008 975 980
113. T. Kitada, Y. Tong, C.A. Gautier, and J. Shen Absence of nigral degeneration in aged parkin/DJ-1/PINK1 triple knockout mice J. Neurochem. 111 2009 696 702
114. S. Kobashigawa, K. Suzuki, and S. Yamashita Ionizing radiation accelerates Drp1-dependent mitochondrial fission, which involves delayed mitochondrial reactive oxygen species production in normal human fibroblast-like cells Biochem. Biophys. Res. Commun 414 2011 795 800
115. W.J. Koopman, S. Verkaart, H.J. Visch, S. van Emst-de Vries, L.G. Nijtmans, J.A. Smeitink, and P.H. Willems Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology? Am. J. Physiol 293 2007 C22 29
116. W.J. Koopman, L.G. Nijtmans, C.E. Dieteren, P. Roestenberg, F. Valsecchi, J.A. Smeitink, and P.H. Willems Mammalian mitochondrial complex I: biogenesis, regulation, and reactive oxygen species generation Antioxid. Redox Signaling 12 2010 1431 1470
117. Y. Kraytsberg, E. Kudryavtseva, A.C. McKee, C. Geula, N.W. Kowall, and K. Khrapko Mitochondrial DNA deletions are abundant and cause functional impairment in aged human substantia nigra neurons Nat. Genet. 38 2006 518 520
118. G. Krebiehl, S. Ruckerbauer, L.F. Burbulla, N. Kieper, B. Maurer, J. Waak, H. Wolburg, Z. Gizatullina, F.N. Gellerich, D. Woitalla, O. Riess, P.J. Kahle, T. Proikas-Cezanne, and R. Krüger Reduced basal autophagy and impaired mitochondrial dynamics due to loss of Parkinsons disease-associated protein DJ-1 PLoS One 5 2010 e9367
119. Y. Kuroda, T. Mitsui, M. Kunishige, M. Shono, M. Akaike, H. Azuma, and T. Matsumoto Parkin enhances mitochondrial biogenesis in proliferating cells Hum. Mol. Genet. 15 2006 883 895 (Pubitemid 43338230)
120. L.L. Lackner, and J. Nunnari Small molecule inhibitors of mitochondrial division: tools that translate basic biological research into medicine Chem. Biol. 17 2010 578 583
121. E. Lamantea, F. Carrara, C. Mariotti, L. Morandi, V. Tiranti, and M. Zeviani A novel nonsense mutation (Q352X) in the mitochondrial cytochrome b gene associated with a combined deficiency of complexes I and III Neuromuscul. Disord. 12 2002 49 52 (Pubitemid 33122151)
122. J. Lapointe, and S. Hekimi When a theory of aging ages badly Cell Mol. Life Sci. 67 2010 1 8
123. H. Latsoudis, C. Spanaki, G. Chlouverakis, and A. Plaitakis Mitochondrial DNA polymorphisms and haplogroups in Parkinsons disease and control individuals with a similar genetic background J. Hum. Genet. 53 2008 349 356
124. M. Lazarou, S.M. Smith, D.R. Thorburn, M.T. Ryan, and M. McKenzie Assembly of nuclear DNA-encoded subunits into mitochondrial complex IV, and their preferential integration into supercomplex forms in patient mitochondria FEBS J 276 2009 6701 6713
125. S.C. Leary, and F. Sasarman Oxidative phosphorylation: synthesis of mitochondrially encoded proteins and assembly of individual structural subunits into functional holoenzyme complexes Methods Mol. Biol 554 2009 143 162
126. Y.J. Lee, S.Y. Jeong, M. Karbowski, C.L. Smith, and R.J. Youle Roles of the mammalian mitochondrial fission and fusion mediators Fis1, Drp1, and Opa1 in apoptosis Mol. Biol. Cell 15 2004 5001 5011 (Pubitemid 39392214)
127. J.J. Lemasters Selective mitochondrial autophagy, or mitophagy, as a targeted defense against oxidative stress, mitochondrial dysfunction, and aging Rejuvenation Res 8 2005 3 5
128. G. Lenaz, C. Bovina, M. DAurelio, R. Fato, G. Formiggini, M.L. Genova, G. Giuliano, M. Merlo Pich, U. Paolucci, G. Parenti Castelli, and B. Ventura Role of mitochondria in oxidative stress and aging Ann. N. Y. Acad. Sci 959 2002 199 213 (Pubitemid 34457563)
129. G. Lenaz, and M.L. Genova Structure and organization of mitochondrial respiratory complexes: a new understanding of an old subject Antioxid. Redox Signaling 12 2010 961 1008
130. O. Levanets, F. Reinecke, R. Louw, P.J. Pretorius, L.H. du Plessis, L. Nijtmans, J.A. Smeitink, and F.H. van der Westhuizen Mitochondrial DNA replication and OXPHOS gene transcription show varied responsiveness to Rieske protein knockdown in 143B cells Biochimie 93 2011 758 765
131. B. Li, Q. Hu, H. Wang, N. Man, H. Ren, L. Wen, N. Nukina, E. Fei, and G. Wang Omi/HtrA2 is a positive regulator of autophagy that facilitates the degradation of mutant proteins involved in neurodegenerative diseases Cell Death Differ. 17 2010 1773 1784
132. Y. Li, M. DAurelio, J.H. Deng, J.S. Park, G. Manfredi, P. Hu, J. Lu, and Y. Bai An assembled complex IV maintains the stability and activity of complex I
133. K.L. Lim, X.H. Ng, L.G.Y. Grace, and T.P. Yao Mitochondrial dynamics and Parkinson's disease: focus on parkin Antioxid. Redox Signaling 16 2012 935 949
134. G. Liot, B. Bossy, S. Lubitz, Y. Kushnareva, N. Sejbuk, and E. Bossy-Wetzel Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway Cell Death Differ. 16 2009 899 909
135. G. Liu, C. Zhang, J. Yin, X. Li, F. Cheng, Y. Li, H. Yang, K. Ueda, P. Chan, and S. Yu alpha-Synuclein is differentially expressed in mitochondria from different rat brain regions and dose-dependently down-regulates complex I activity Neurosci. Lett. 454 2009 187 192
136. X. Liu, L. Feng, M. Yan, K. Xu, Y. Yu, and X. Zheng Changes in mitochondrial dynamics during amyloid β-induced PC12 cell apoptosis Mol. Cell. Biochem. 344 2010 277 284
137. V. Loeb, E. Yakunin, A. Saada, and R. Sharon The transgenic overexpression of alpha-synuclein and not its related pathology associates with complex I inhibition J. Biol. Chem. 285 2010 7334 7343
138. A. Ludlam, J. Brunzelle, T. Pribyl, X. Xu, D.L. Gatti, and S.H. Ackerman Chaperones of F1-ATPase J. Biol. Chem. 284 2009 17138 17146
139. J.W. Lustbader, M. Cirilli, C. Lin, H.W. Xu, K. Takuma, N. Wang, C. Caspersen, X. Chen, S. Pollak, M. Chaney, F. Trinchese, S. Liu, F. Gunn-Moore, L.F. Lue, D.G. Walker, P. Kuppusamy, Z.L. Zewier, O. Arancio, D. Stern, S.S. Yass, and H. Wu ABAD directly links Abeta to mitochondrial toxicity in Alzheimers disease Science 304 2004 448 452 (Pubitemid 38495942)
140. M. Mancuso, M. Nardini, D. Micheli, A. Rocchi, C. Nesti, N.J. Giglioli, L. Petrozzi, C Rossi, R. Ceravolo, A. Bacci, A. Choub, G. Ricci, G. Tognoni, M.L. Manca, G. Siciliano, and L. Murri Lack of association between mtDNA haplogroups and Alzheimers disease in Tuscany Neurol. Sci. 28 2007 142 147 (Pubitemid 47039841)
141. M. Manczak, T.S. Anekonda, E. Henson, B.S. Park, J. Quinn, and P.H. Reddy Mitochondria are a direct site of A beta accumulation in Alzheimers disease neurons: implications for free radical generation and oxidative damage in disease progression Hum. Mol. Genet. 15 2006 1437 1449
142. M. Manczak, M.J. Calkins, and P.H. Reddy Impaired mitochondrial dynamics and abnormal interaction of amyloid beta with mitochondrial protein Drp1 in neurons from patients with Alzheimers disease: implications for neuronal damage Hum. Mol. Genet 20 2011 2495 2509
143. A. Maruszak, J.A. Canter, M. Styczyńska, C. Zekanowski, and M. Barcikowska Mitochondrial haplogroup H and Alzheimers disease - is there a connection? Neurobiol. Aging 30 2009 1749 1755
144. V. Massa, E. Fernandez-Vizarra, S. Alshahwan, E. Bakhsh, P. Goffrini, I. Ferrero, P. Mereghetti, P. DAdamo, P. Gasparini, and M. Zeviani Severe infantile encephalomyopathy caused by a mutation in COX6B1, a nucleus-encoded subunit of cytochrome c oxidase Am. J. Hum. Genet. 82 2008 1281 1289
145. D. Matenia, C. Hempp, T. Timm, A. Eikhof, and E.M. Mandelkow Microtubule affinity-regulating kinase 2 (MARK2) turns on phosphatase and tensin homolog (PTEN)-induced kinase 1 (PINK1) at Thr-313, a mutation site in Parkinsons disease: effects on mitochondrial transport J. Biol. Chem 287 2012 8174 8186
146. N. Matsuda, S. Sato, K. Shiba, K. Okatsu, K. Saisho, C.A. Gautier, Y.S. Sou, S. Saiki, S. Kawajiri, F. Sato, M. Kimura, M. Komatsu, N. Hattori, and K. Tanaka PINK1 stabilized by mitochondrial depolarization recruits parkin to damaged mitochondria and activates latent parkin for mitophagy J. Cell Biol. 189 2010 211 221
147. M. McKenzie, and M.T. Ryan Assembly factors of human mitochondrial complex I and their defects in disease IUBMB Life 62 2010 497 502
148. P. Mecocci, U. MacGarvey, and M.F. Beal Oxidative damage to mitochondrial DNA is increased in Alzheimers disease Ann. Neurol. 36 1994 747 751 (Pubitemid 24337634)
149. P. Mehta, G.D. Mellick, D.B. Rowe, G.M. Halliday, M.M. Jones, N. Manwaring, H. Vandebona, P.A. Silburn, J.J. Wang, P. Mitchell, and C.M. Sue Mitochondrial DNA haplogroups J and K are not protective for Parkinsons disease in the Australian community Mov. Disord 24 2009 290 292
150. P. Mitchell, and J. Moyle Respiration-driven proton translocation in rat liver mitochondria Biochem. J. 105 1967 1147 1162
151. K. Mitra, C. Wunder, B. Roysam, G. Lin, and J. Lippincott-Schwartz A hyperfused mitochondrial state achieved at G1-S regulates cyclin E buildup and entry into S phase Proc. Ann. Acad. Sci 106 2009 11960 11965
152. V.A. Morais, and B. De Strooper Mitochondria dysfunction and neurodegenerative disorders: cause or consequence J. Alzheimers Dis 20 Suppl. 2 2010 S255 263
153. M. Moran, L. Marin-Buera, M.C. Gil-Borlado, H. Rivera, A. Blazquez, S. Seneca, M. Vazquez-Lopez, J. Arenas, M.A. Martin, and C. Ugalde Cellular pathophysiological consequences of BCS1L mutations in mitochondrial complex III enzyme deficiency Hum. Mutat. 31 2010 930 941
154. M. Moran, H. Rivera, M. Sanchez-Arago, A. Blazquez, B. Merinero, C. Ugalde, J. Arenas, J.M. Cuezva, and M.A. Martin Mitochondrial bioenergetics and dynamics interplay in complex I-deficient fibroblasts Biochim. Biophys. Acta 1802 2010 443 453
155. D. Moreno-Lastres, F. Fontanesi, I. García-Consuegra, M.A. Martín, J. Arenas, A. Barrientos, and C. Ugalde Mitochondrial complex I plays an essential role in human respirasome assembly Cell Metab. 15 2012 324 335
156. H. Mortiboys, K.J. Thomas, W.J. Koopman, S. Klaffke, P. Abou-Sleiman, S. Olpin, N.W. Wood, P.H. Willems, J.A. Smeitink, M.R. Cookson, and O. Bandmann Mitochondrial function and morphology are impaired in parkin-mutant fibroblasts Ann. Neurol. 64 2008 555 565
157. H. Mortiboys, K.K. Johansen, J.O. Aasly, and O. Bandman Mitochondrial impairment in patients with Parkinson disease with the G2019S mutation in LRRK2 Neurology 75 2010 2017 2020
158. M.P. Murphy How mitochondria produce reactive oxygen species Biochem. J. 417 2009 1 13
159. K. Nakada, K. Inoue, T. Ono, K. Isobe, A. Ogura, Y.I. Goto, I. Nonaka, and J.I. Hayashi Inter-mitochondrial complementation: mitochondria-specific system preventing mice from expression of disease phenotypes by mutant mtDNA Nat. Med 7 2001 934 940 (Pubitemid 32756431)
160. D. Narendra, A. Tanaka, D.F. Suen, and R.J. Youle Parkin is recruited selectively to impaired mitochondria and promotes their autophagy J. Cell Biol. 183 2008 795 803
161. D.P. Narendra, S.M. Jin, A. Tanaka, D.F. Suen, C.A. Gautier, J. Shen, M.R. Cookson, and R.J. Youle PINK1 is selectively stabilized on impaired mitochondria to activate Parkin PLoS Biol. 8 2010 e1000298
162. L.C. Papadopoulou, C.M. Sue, M.M. Davidson, K. Tanji, I. Nishino, J.E. Sadlock, S. Krishna, W. Walker, J. Selby, D.M. Glerum, R.V. Coster, G. Lyon, E. Scalais, R. Lebel, P. Kaplan, S. Shanske, D.C. De Vivo, E. Bonilla, M. Hirano, S. DiMauro, and E.A. Schon Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene Nat. Genet. 23 1999 333 337 (Pubitemid 29526433)
163. M.S. Parihar, M. Parihar, M. Fujita, P. Hashimoto, and P. Ghafourifar Mitochondrial association of alpha-synuclein causes oxidative stress Cell Mol. Life Sci. 65 2008 1272 1284
164. J. Park, J. Lee, and C. Choi Mitochondrial network determines intracellular ROS dynamics and sensitivity to oxidative stress through switching inter-mitochondrial messengers PLoS One 6 2011 e23211
165. J.K. Parks, T.S. Smith, P.A. Trimmer, J.P. Bennett Jr., and W.D. Parker Jr. Neurotoxic Abeta peptides increase oxidative stress in vivo through NMDA-receptor and nitric-oxide-synthase mechanisms, and inhibit complex IV activity and induce a mitochondrial permeability transition in vitro J. Neurochem. 76 2001 1050 1066
166. D.A. Patten, M. Germain, M.A. Kelly, and R.S. Slack Reactive oxygen species: stuck in the middle of neurodegeneration J. Alzheimers Dis 20 Suppl. 2 2010 S357 367
167. P.F. Pavlov, B. Wiehager, J. Sakai, S. Fryman, H. Behbahani, B. Winblad, and M. Ankarcrona Mitochondrial γ-secretase participates in the metabolism of mitochondria-associated amyloid precursor protein FASEB J 25 2011 78 88
168. R. Pello, M.A. Martin, V. Carelli, L.G. Nijtmans, A. Achilli, M. Pala, A. Torroni, A. Gomez-Duran, E. Ruiz-Pesini, A. Martinuzzi, J.A. Smeitink, J. Arenas, and C. Ugalde Mitochondrial DNA background modulates the assembly kinetics of OXPHOS complexes in a cellular model of mitochondrial disease Hum. Mol. Genet. 17 2008 4001 4011 (Pubitemid 352762860)
169. K. Pennington, J. Peng, C.C. Hung, R.E. Banks, and P.A. Robinson Differential effects of wild-type and A53T mutant isoform of alpha-synuclein on the mitochondrial proteome of differentiated SH-SY5Y cells J. Proteome Res. 9 2010 2390 2401
170. N.A. Pham, T. Richardson, J. Cameron, B. Chue, and B.H. Robinson Altered mitochondrial structure and motion dynamics in living cells with energy metabolism defects revealed by real time microscope imaging Microsc. Microanal. 10 2004 247 260 (Pubitemid 38531947)
171. C. Piccoli, A. Sardanelli, R. Scrima, M. Ripoli, G. Quarato, A. DAprile, F. Bellomo, S. Scacco, G. De Michele, A. Filla, A. Iuso, D. Boffoli, N. Capitanio, and S. Papa Mitochondrial respiratory dysfunction in familiar parkinsonism associated with PINK1 mutation Neurochem. Res. 33 2008 2565 2574
172. S. Pitkanen, and B.H. Robinson Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase J. Clin. Invest 98 1996 345 351 (Pubitemid 26255732)
173. O.Y. Pletjushkina, K.G. Lyamzaev, E.N. Popova, O.K. Nepryakhina, O.Y. Ivanova, L.V. Domnina, B.V. Chernyak, and V.P. Skulachev Effect of oxidative stress on dynamics of mitochondrial reticulum Biochim. Biophys. Acta 1757 2006 518 524 (Pubitemid 43996946)
174. R.A. Quintanilla, T.A. Matthews/Robertson, P.J. Dolan, and G.V. Johnson Caspase-cleaved tau expression induces mitochondrial dysfunction in immortalized cortical neurons, implications for the pathogenesis of Alzheimer disease J. Biol. Chem. 284 2009 18754/18766
175. R.A. Quintanilla, P.J. Dolan, Y.N. Jin, Y.N. Jin, and G.V.W. Johnson Truncated tau and Aβ cooperatively impair mitochondria in primary neurons Neurobiol. Aging 33 2012 e25 35
176. S. Rahman, J.W. Taanman, J.M. Cooper, I. Nelson, I. Hargreaves, B. Meunier, M.G. Hanna, J.J. García, R.A. Capaldi, B.D. Lake, J.V. Leonard, and A.H. Schapira A missense mutation of cytochrome oxidase subunit II causes defective assembly and myopathy Am. J. Hum. Genet. 65 1999 1030 1039 (Pubitemid 30462959)
177. C.P. Ramsey, and B.I. Giasson The E163K DJ-1 mutant shows specific antioxidant deficiency Brain Res. 1239 2008 1 11
178. M. Rana, I. de Coo, F. Diaz, H. Smeets, and C.T. Moraes An out-of-frame cytochrome b gene deletion from a patient with parkinsonism is associated with impaired complex III assembly and an increase in free radical production Ann. Neurol 48 2000 774 781
179. R.A. Reid, J. Moyle, and P. Mitchell Synthesis of adenosine triphosphate by a protonmotive force in rat liver mitochondria Nature 212 1966 257 258
180. V. Rhein, X. Song, A. Wiesner, L.M. Ittner, G. Baysang, F. Meier, L. Ozmen, H. Bluethmann, S. Drose, U. Brandt, E. Savaskan, C. Czech, J. Gotz, and A. Eckert Amyloid-β and tau synergistically impairs the oxidative phosphorylation system in triple transgenic Alzheimers disease mice Proc. Natl. Acad. Sci. USA 106 2009 20057 20062
181. S. Rodriguez-Enriquez, L. He, and J.J. Lemasters Role of mitochondrial permeability transition pores in mitochondrial autophagy Int. J. Biochem. Cell Biol. 36 2004 2463 2472 (Pubitemid 39119759)
182. D.C. Rubinsztein, G. Mariño, and G. Kroemer Autophagy and aging Cell 146 2011 682 695
183. J. Rutter, D.R. Winge, and J.D. Schiffman Succinate dehydrogenase - assembly, regulation and role in human disease Mitochondrion 10 2010 393 401
184. M.T. Ryan, and N.J. Hoogenraad Mitochondrial-nuclear communications Annu. Rev. Biochem. 76 2007 701 722
185. A. Saada, S. Edvardson, A. Shaag, W.K. Chung, R. Segel, C. Miller, C. Jalas, and O. Elpeleg Combined OXPHOS complex I and IV defect, due to mutated complex I assembly factor C20ORF7 J. Inherit. Metab. Dis. 35 2011 125 131
186. S. Saddar, M.K. Dienhart, and R.A. Stuart The F1F0-ATP synthase complex influences the assembly state of the cytochrome bc1-cytochrome oxidase supercomplex and its association with the TIM23 machinery J. Biol. Chem 283 2008 6677 6686
187. G. Sala, F. Trombin, S. Beretta, L. Tremolizzo, P. Presutto, M. Montopoli, M. Fantin, A. Martinuzzi, V. Carelli, and C. Ferrarese Antioxidants partially restore glutamate transport defect in Leber hereditary optic neuropathy cybrids J. Neurosci. Res. 86 2008 3331 3337
188. A. Santoro, V. Balbi, E. Balducci, C. Pirazzini, F. Rosini, F. Tavano, A. Achilli, P. Siviero, N. Minicuci, E. Bellavista, M. Mishto, S. Salvioli, F. Marchegiani, M. Cardelli, F. Olivieri, B. Nacmias, A.M. Chiamenti, L. Benussi, R. Ghidoni, G. Rose, C. Gabelli, G. Binetti, S. Sorbi, G. Crepaldi, G. Passarino, A. Torroni, and C. Franceschi Evidence for sub-haplogroup h5 of mitochondrial DNA as a risk factor for late onset Alzheimers disease PLoS One 5 2010 e12037
189. C. Sauvanet, S. Duvezin-Caubet, J.P. di Rago, and M. Rojo Energetic requirements and bioenergetic modulation of mitochondrial morphology and dynamics Semin. Cell Dev. Biol. 21 2010 558 565
190. H. Schagger, and K. Pfeiffer Supercomplexes in the respiratory chains of yeast and mammalian mitochondria EMBO J 19 2000 1777 1783 (Pubitemid 30204389)
191. H. Schagger, and K. Pfeiffer The ratio of oxidative phosphorylation complexes I-V in bovine heart mitochondria and the composition of respiratory chain supercomplexes J. Biol. Chem 276 2001 37861 37867
192. H. Schagger Respiratory chain supercomplexes of mitochondria and bacteria Biochim. Biophys. Acta 1555 2002 154 159 (Pubitemid 35246021)
193. H. Schagger, R. de Coo, M.F. Bauer, S. Hofmann, C. Godinot, and U. Brandt Significance of respirasomes for the assembly/stability of human respiratory chain complex I J. Biol. Chem. 279 2004 36349 36353 (Pubitemid 39128972)
194. E.A. Schon, S. DiMauro, M. Hirano, and R.W. Gilkerson Therapeutic prospects for mitochondrial disease Trends Mol. Med. 16 2010 268 276
195. H. Seelert, D.N. Dani, S. Dante, T. Hauss, F. Krause, E. Schäfer, M. Frenzel, A. Poetsch, S. Rexroth, H.J. Schwassmann, T. Suhai, J. Vonck, and N.A. Dencher From protons to OXPHOS supercomplexes and Alzheimers disease: structure-dynamics-function relationships of energy-transducing membranes Biochim. Biophys. Acta 1787 2009 657 671
196. M.K. Shigenaga, T.M. Hagen, and B.N. Ames Oxidative damage and mitochondrial decay in aging Proc. Natl. Acad. Sci. USA 91 1994 10771 10778 (Pubitemid 24349567)
197. J.H. Shin, H.S. Ko, H. Kang, Y. Lee, Y.I. Lee, O. Pletinkkova, J.C. Troconso, V.L. Dawson, and T.M. Dawson PARIS(ZNF746) repression of PGC-1α contributes to neurodegeneration in Parkinsons disease Cell 144 2011 689 702
198. D.F. Silva, A.R. Esteves, D.M. Arduino, C.R. Oliveira, and S.M. Cardoso Amyloid-β-induced mitochondrial dysfunction impairs the autophagic lysosomal pathway in a tubulin dependent pathway J. Alzheimers Dis. 26 2011 565 581
199. D.K. Simon, N. Pankratz, D.K. Kissell, M.W. Pauciulo, C.A. Halter, A. Rudolph, R.F. Pfeiffer, W.C. Nichols, and T. Foroud Maternal inheritance and mitochondrial DNA variants in familial Parkinsons disease BMC Med. Genet. 11 2010 53
200. V.P. Skulachev Mitochondrial filaments and clusters as intracellular power-transmitting cables Trends Biochem. Sci. 26 2001 23 29 (Pubitemid 32124706)
201. P.M. Smith, J.L. Fox, and D.R. Winge Biogenesis of the cytochrome bc(1) complex and role of assembly factors Biochim. Biophys. Acta 1817 2012 276 286
202. I.C. Soto, F. Fontanesi, M. Valledor, D. Horn, R. Singh, and A. Barrientos Synthesis of cytochrome c oxidase subunit 1 is translationally downregulated in the absence of functional F1F0-ATP synthase Biochim. Biophys. Acta 1793 2009 1776 1786
203. M.G. Spillantini, R.A. Crowther, R. Jakes, N.J. Cairns, P.L. Lantos, and M. Goedert Filamentous alpha-synuclein inclusions link multiple system atrophy with Parkinsons disease and dementia with Lewy bodies Neurosci. Lett. 251 1998 205 208 (Pubitemid 28381082)
204. M.G. Spillantini, R.A. Crowther, R. Jakes, M. Hasegawa, and M. Goedert Alpha-Synuclein in filamentous inclusions of Lewy bodies from Parkinsons disease and dementia with Lewy bodies Proc. Natl. Acad. Sci. USA 95 1998 6469 6473 (Pubitemid 28249037)
205. L. Stiburek, and J. Zeman Assembly factors and ATP-dependent proteases in cytochrome c oxidase biogenesis Biochim. Biophys. Acta 1797 2010 1149 1158
206. D.F. Suen, K.L. Norris, and R.J. Youle Mitochondrial dynamics and apoptosis Genes Dev. 22 2008 1577 1590 (Pubitemid 351846838)
207. S. Suissa, Z. Wang, J. Poole, S. Wittkopp, J. Feder, T.E. Shutt, D.C. Wallace, G.S. Shadel, and D. Mishmar Ancient mtDNA genetic variants modulate mtDNA transcription and replication PLoS Genet. 5 2009 e1000474
208. N. Taguchi, N. Ishihara, A. Jofuku, T. Oka, and K. Mihara Mitotic phosphorylation of dynamin-related GTPase Drp1 participates in mitochondrial fission J. Biol. Chem. 282 2007 11521 11529 (Pubitemid 47100787)
209. A. Tanaka, S. Kobayashi, and Y. Fujiki Peroxisome division is impaired in a CHO cell mutant with an inactivating point-mutation in dynamin-like protein 1 gene Exp. Cell Res 312 2006 1671 1684
210. K.J. Thomas, M.K. McCoy, J. Blackinton, A. Beilina, M. van der Brug, A. Sandebring, D. Miller, D. Maric, A. Cedazo-Minguez, and M.R. Cookson DJ-1 acts in parallel to the PINK1/parkin pathway to control mitochondrial function and autophagy Hum. Mol. Genet. 20 2011 40 50
211. V. Tiranti, K. Hoertnagel, R. Carrozzo, C. Galimberti, M. Munaro, M. Granatiero, L. Zelante, P. Gasparini, R. Marzella, M. Rocchi, M.P. Bayona-Bafaluy, J.A. Enriquez, G. Uziel, E. Bertini, C. Dionisi-Vici, B. Franco, T. Meitinger, and M. Zeviani Mutations of SURF-1 in Leigh disease associated with cytochrome c oxidase deficiency Am. J. Hum. Genet. 63 1998 1609 1621 (Pubitemid 30415723)
212. D. Tondera, S. Grandemange, A. Jourdain, M. Karbowski, Y. Mattenberger, S. Herzing, S. Da Cruz, P. Clerc, I. Raschke, C. Merkwith, S. Ehses, F. Krause, D.C. Chan, C. Alexander, C. Bauer, R. Youle, T. Langer, and J-C. Martinou SL-2 is required for stress-induced mitochondrial hyperfusion EMBO J 28 2009 1589 1600
213. A. Torroni, M. Petrozzi, L. DUrbano, D. Sellitto, M. Zeviani, F. Carrara, C. Carducci, V. Leuzzi, V. Carelli, P. Barboni, A. De Negri, and R. Scozzari 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 1997 1107 1121 (Pubitemid 27194095)
214. J.F. Turrens Mitochondrial formation of reactive oxygen species J. Physiol. 552 2003 335 344 (Pubitemid 37321833)
215. G. Twig, A. Elorza, A.J. Molina, H. Mohamed, J.D. Wikstrom, G. Walzer, L. Stiles, S.E. Haigh, S. Katz, G. Las, J. Alroy, M. Wu, B.F. Py, J. Yuan, J.T. Deeney, B.E. Corkey, and O. Shirihai Fission and selective fusion govern mitochondrial segregation and elimination by autophagy EMBO J 27 2008 433 446 (Pubitemid 351161653)
216. G. Twig, B. Hyde, and O.S. Shirihai Mitochondrial fusion, fission and autophagy as a quality control axis: the bioenergetic view Biochim. Biophys. Acta 1777 2008 1092 1097
217. C. Ugalde, R.J. Janssen, L.P. van den Heuvel, J.A. Smeitink, and L.G. Nijtmans Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency Hum. Mol. Genet. 13 2004 659 667 (Pubitemid 38379855)
218. C. Ugalde, M. Morán, A. Blazquez, J. Arenas, and M.A. Martin Mitochondrial disorders due to nuclear OXPHOS gene defects Adv. Exp. Med. Biol. 652 2009 85 116
219. I. Valnot, S. Osmond, N. Gigarel, B. Mehaye, J. Amiel, V. Cormier-Daire, A. Munnich, J.P. Bonnefont, P. Rustin, and A. Rötig Mutations of the SCO1 gene in mitochondrial cytochrome c oxidase deficiency with neonatal-onset hepatic failure and encephalopathy Am. J. Hum. Genet. 67 2000 1104 1109
220. I. Valnot, J.C. von Kleist-Retzow, A. Barrientos, M. Gorbatyuk, J.W. Taanman, B. Mehaye, P. Rustin, A. Tzagoloff, A. Munnich, and A. Rötig A mutation in the human heme A:farnesyltransferase gene (COX10) causes cytochrome c oxidase deficiency Hum. Mol. Genet. 9 2000 1245 1249 (Pubitemid 30248611)
221. V.S. Van Laar, B. Arnold, S.J. Cassady, C.T. Chu, E.A. Burton, and S.B. Berman Bioenergetics of neurons inhibit the translocation response of parkin following rapid mitochondrial depolarization Hum. Mol. Genet. 20 2011 927 940
222. T. Vargas, C. Ugalde, C. Spuch, D. Antequera, M.J. Morán, M.A. Martin, I. Ferrer, F. Bermejo-Pareja, and E. Carro Abeta accumulation in choroid plexus is associated with mitochondrial-induced apoptosis Neurobiol. Aging 31 2010 1569 1581
223. U.D. Vempati, X. Han, and C.T. Moraes Lack of cytochrome c in mouse fibroblasts disrupts assembly/stability of respiratory complexes I and IV J. Biol. Chem. 284 2009 4383 4391
224. S. Verkaart, W.J. Koopman, J. Cheek, S.E. van Emst-de Vries, L.W. van den Heuvel, J.A. Smeitink, and P.H. Willems Mitochondrial and cytosolic thiol redox state are not detectably altered in isolated human NADH:ubiquinone oxidoreductase deficiency Biochim. Biophys. Acta 1772 2007 1041 1051 (Pubitemid 47314228)
225. S. Verkaart, W.J. Koopman, S.E. van Emst-de Vries, L.G. Nijtmans, L.W. van den Heuvel, J.A. Smeitink, and P.H. Willems Superoxide production is inversely related to complex I activity in inherited complex I deficiency Biochim. Biophys. Acta 1772 2007 373 381 (Pubitemid 46275438)
226. S. Vilain, G. Esposito, D. Haddad, O. Schaap, M.P. Dobreva, M. Vos, S. Van Meensel, V.A. Morais, B. De Strooper, and P. Verstreken The yeast complex I equivalent NADH dehydrogenase rescues pink1 mutants PLoS Genet. 8 2012 e1002456
227. C. Vives-Bauza, R. Gonzalo, G. Manfredi, E. Garcia-Arumi, and A.L. Andreu Enhanced ROS production and antioxidant defenses in cybrids harbouring mutations in mtDNA Neurosci. Lett. 391 2006 136 141 (Pubitemid 41736187)
228. C. Vives-Bauza, C. Zhou, Y. Huang, M. Cui, R.L. de Vries, J. Kim, J. May, M.A. Tocilescu, W. Liu, H.S. Ko, J. Magrané, D.J. Moore, V.L. Dawson, R. Grailhe, T.M. Dawson, C. Li, K. Tieu, and S. Przedborski PINK1-dependent recruitment of parkin to mitochondria in mitophagy Proc. Natl. Acad. Sci. USA 107 2010 378 383
229. K.A. Vossel, K. Zhang, J. Brodbeck, A.C. Daub, P. Sharma, S. Finkbeiner, B. Cui, and L. Mucke Tau reduction prevents Abeta-induced defects in axonal transport Science 330 2010 198 201
230. J. Wang, S. Xiong, C. Xie, W.R. Markesbery, and M.A. Lovell Increased oxidative damage in nuclear and mitochondrial DNA in Alzheimers disease J. Neurochem. 93 2005 953 962 (Pubitemid 40695754)
231. M.S. Wang, S. Boddapati, S. Emadi, and M.R. Sierks Curcumin reduces alpha-synuclein induced cytotoxicity in Parkinsons disease cell model BMC Neurosci 11 2010 57
232. X. Wang, B. Su, S.L. Siedlak, P.I. Moreira, H. Fujioka, Y. Wang, G. Casadesus, and X. Zhu Amyloid-beta overproduction causes abnormal mitochondrial dynamics via differential modulation of mitochondrial fission/fusion proteins Proc. Natl. Acad. Sci. USA 105 2008 19318 19323
233. X. Wang, B. Su, H.G. Lee, X. Li, G. Perry, M.A. Smith, and X. Zhu Impaired balance of mitochondrial fission and fusion in Alzheimers disease J. Neurosci. 29 2009 9090 9103
234. X. Wang, D. Winter, G. Ashrafi, J. Schlehe, Y.L. Wong, D. Selkoe, S. Rice, J. Steen, M.J. LaVoie, and T.L. Schwarz PINK1 and parkin target Miro for phosphorylation and degradation to arrest mitochondrial motility Cell 147 2011 893 906
235. T.A. Weber, and A.S. Reichert Impaired quality control of mitochondria: aging from a new perspective Exp. Gerontol. 45 2010 503 511
236. P.H. Willems, J.A. Smeitink, and W.J. Koopman Mitochondrial dynamics in human NADH:ubiquinone oxidoreductase deficiency Int. J. Biochem. Cell Biol. 41 2009 1773 1782
237. I. Wittig, and H. Schagger Supramolecular organization of ATP synthase and respiratory chain in mitochondrial membranes Biochim. Biophys. Acta 1787 2009 672 680
238. A. Wong, L. Cavelier, H.E. Collins-Schramm, M.F. Seldin, M. McGrogan, M.L. Savontaus, and G.A. Cortopassi Differentiation-specific effects of LHON mutations introduced into neuronal NT2 cells Hum. Mol. Genet. 11 2002 431 438 (Pubitemid 34213616)
239. W. Xie, O.W. Wan, and K.K. Chung New insights into the role of mitochondrial dysfunction and protein aggregation in Parkinsons disease Biochim. Biophys. Acta 1802 2010 935 941
240. S.Y. Yang, X.Y. He, and D. Miller HSD17B10: a gene involved in cognitive function through metabolism of isoleucine and neuroactive steroids Mol. Genet. Metab. 92 36-42 2007 2007
241. J. Yao, M. Taylor, F. Davey, Y. Ren, J. Aiton, P. Coote, F. Fang, J.X. Chen, S.D. Yan, and F.J. Gunn-Moore Interaction of amyloid binding alcohol dehydrogenase/Abeta mediates up-regulation of peroxiredoxin II in the brains of Alzheimers disease patients and a transgenic Alzheimers disease mouse model Mol. Cell. Neurosci. 35 2007 377 382 (Pubitemid 46843572)
242. J. Yao, H. Du, S. Yan, F. Fang, C. Wang, L.F. Lue, L. Guo, D. Chen, D.M. Stern, F.J. Gunn Moore, C.J Xi, O. Arancio, and S.S. Yan Inhibition of amyloid-beta (Abeta) peptide-binding alcohol dehydrogenase-Abeta interaction reduces Abeta accumulation and improves mitochondrial function in a mouse model of Alzheimers disease J. Neurosci. 31 2011 2313 2320
243. M.Y. Yen, A.G. Wang, and Y.H. Wei Lebers hereditary optic neuropathy: a multifactorial disease Prog. Retinal Eye Res 25 2006 381 396
244. W.L. Yen, and D.J. Klionsky How to live long and prosper: autophagy, mitochondria, and aging Physiology (Bethesda) 23 2008 248 262
245. Y.S. Yoon, D.S. Yoon, I.K. Lim, S.H. Yoon, H.Y. Chung, M. Rojo, F. Malka, M.J. Jou, J.C. Martinou, and G. Yoon Formation of elongated giant mitochondria in DFO-induced cellular senescence: involvement of enhanced fusion process through modulation of Fis1 J. Cell. Physiol. 209 2006 468 480 (Pubitemid 44484638)
246. T. Yu, J.L. Robotham, and Y. Yoon Increased production of reactive oxygen species in hyperglycemic conditions requires dynamic change of mitochondrial morphology Proc. Natl. Acad. Sci. USA 103 2006 2653 2658
247. T. Yu, S.S. Sheu, J.L. Robotham, and Y. Yoon Mitochondrial fission mediates high glucose-induced cell death through elevated production of reactive oxygen species Cardiovasc. Res. 79 2008 341 351 (Pubitemid 351951612)
248. T. Yu, B.S. Jhun, and Y. Yoon High-glucose stimulation increases reactive oxygen species production through the calcium and mitogen-activated protein kinase-mediated activation of mitochondrial fission Antioxid. Redox Signaling 14 2011 425 437
249. V. Zara, L. Conte, and B.L. Trumpower Biogenesis of the yeast cytochrome bc1 complex Biochim. Biophys. Acta 1793 2009 89 96
250. L. Zhang, L. Yu, and C. Yu Generation of superoxide anion by succinate-cytochrome c reductase from bovine heart mitochondria J. Biol. Chem. 273 1998 33972 33976 (Pubitemid 29008862)
251. C. Zhou, Y. Huang, and S. Przedborski Oxidative stress in Parkinsons disease: a mechanism of pathogenic and therapeutic significance Ann. N. Y. Acad. Sci 1147 2008 93 104
252. J. Zhu, and C.T. Chu Mitochondrial dysfunction in Parkinsons disease J. Alzheimers Dis 20 Suppl. 2 2010 S325 334
253. Z. Zhu, J. Yao, T. Johns, K. Fu, I. De Bie, C. Macmillan, A.P. Cuthbert, R.F. Newbold, J. Wang, M. Chevrette, G.K. Brown, R.M. Brown, and E.A. Shoubridge SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome Nat. Genet. 20 1998 337 343 (Pubitemid 28541630)
254. D.B. Zorov, C.R. Filburn, L.O. Klotz, J.L. Zweier, and S.J. Sollott Reactive oxygen species (ROS)-induced ROS release: a new phenomenon accompanying induction of the mitochondrial permeability transition in cardiac myocytes J. Exp. Med. 192 2000 1001 1014
255. S. Zuchner, I.V. Mersiyanova, M. Muglia, N. Bissar-Tadmouri, J. Rochelle, E.L. Dadali, M. Zappia, E. Nelis, A. Patitucci, J. Senderek, Y. Parman, O. Evgrafov, P.D. Jonghe, Y. Takahashi, S. Tsui., M.A. Pericak-Vance, A. Quattrone, E. Battaloglu, A.V. Polyakov, V. Timmerman, J.M. Schröder, and J.M. Vance Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2 A Nat. Genet. 36 2004 449 451 (Pubitemid 38620027)
256. S.J. Chinta, M.J. Kumar, M. Hsu, S. Rajagopalan, D. Kaur, A. Rane, D.G. Nicholls, J. Choi, and J.K. Andersen Inducible alterations of glutathione levels in adult dopaminergic midbrain neurons result in nigrostratal degeneration J. Neurosci. 27 51 2007 13997 14006 (Pubitemid 351377875)