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1

Schägger, Hermann. "Respiratory Chain Supercomplexes." IUBMB Life (International Union of Biochemistry and Molecular Biology: Life) 52, no. 3-5 (September 1, 2001): 119–28. http://dx.doi.org/10.1080/15216540152845911.

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2

Ameyama, Minoru, Kazunobu Matsushita, Emiko Shinagawa, and Osao Adachi. "Sugar-oxidizing Respiratory Chain ofGluconobacter suboxydans. Evidence for a Branched Respiratory Chain and Characterization of Respiratory Chain-Linked Cytochromes." Agricultural and Biological Chemistry 51, no. 11 (November 1987): 2943–50. http://dx.doi.org/10.1080/00021369.1987.10868527.

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3

DiMauro, Salvatore, and Eric A. Schon. "Mitochondrial Respiratory-Chain Diseases." New England Journal of Medicine 348, no. 26 (June 26, 2003): 2656–68. http://dx.doi.org/10.1056/nejmra022567.

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4

Rich, Peter R., and Amandine Maréchal. "The mitochondrial respiratory chain." Essays in Biochemistry 47 (June 14, 2010): 1–23. http://dx.doi.org/10.1042/bse0470001.

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In the present chapter, the structures and mechanisms of the major components of mammalian mitochondrial respiratory chains are reviewed. Particular emphasis is placed on the four protein complexes and their cofactors that catalyse the electron transfer pathway between oxidation of NADH and succinate and the reduction of oxygen to water. Current ideas are reviewed of how these electron transfer reactions are coupled to formation of the proton and charge gradient across the inner mitochondrial membrane that is used to drive ATP synthesis. Additional respiratory components that are found in mammalian and plant, fungal and algal mitochondria are also reviewed.
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5

AMEYAMA, Minoru, Kazunobu MATSUSHITA, Emiko SHINAGAWA, and Osao ADACHI. "Sugar-oxidizing respiratory chain of Gluconobacter suboxydans. Evidence for a branched respiratory chain and characterization of respiratory chain-linked cytochromes." Agricultural and Biological Chemistry 51, no. 11 (1987): 2943–50. http://dx.doi.org/10.1271/bbb1961.51.2943.

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6

MBBS, Joannie Hui, Denise M. Kirby, David R. Thorburn, and A. vihu Boneh. "Decreased activities of mitochondrial respiratory chain complexes in non-mitochondrial respiratory chain diseases." Developmental Medicine & Child Neurology 48, no. 2 (February 2006): 132–36. http://dx.doi.org/10.1017/s0012162206000284.

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7

Meuric, Vincent, Astrid Rouillon, Fatiha Chandad, and Martine Bonnaure-Mallet. "Putative respiratory chain ofPorphyromonas gingivalis." Future Microbiology 5, no. 5 (May 2010): 717–34. http://dx.doi.org/10.2217/fmb.10.32.

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8

Shoubridge, Eric A. "Supersizing the Mitochondrial Respiratory Chain." Cell Metabolism 15, no. 3 (March 2012): 271–72. http://dx.doi.org/10.1016/j.cmet.2012.02.009.

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9

Pfeiffer, Kathy, Vishal Gohil, Rosemary A. Stuart, Carola Hunte, Ulrich Brandt, Miriam L. Greenberg, and Hermann Schägger. "Cardiolipin Stabilizes Respiratory Chain Supercomplexes." Journal of Biological Chemistry 278, no. 52 (October 15, 2003): 52873–80. http://dx.doi.org/10.1074/jbc.m308366200.

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10

Zhang, Mei, Eugenia Mileykovskaya, and William Dowhan. "Gluing the Respiratory Chain Together." Journal of Biological Chemistry 277, no. 46 (October 2, 2002): 43553–56. http://dx.doi.org/10.1074/jbc.c200551200.

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11

Guenthard, J., F. Wyler, B. Fowler, and R. Baumgartner. "Cardiomyopathy in respiratory chain disorders." Archives of Disease in Childhood 72, no. 3 (March 1, 1995): 223–26. http://dx.doi.org/10.1136/adc.72.3.223.

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12

Triepels, R. H., L. P. Van Den Heuvel, J. M. Trijbels, and J. A. Smeitink. "Respiratory chain complex I deficiency." American Journal of Medical Genetics 106, no. 1 (2001): 37–45. http://dx.doi.org/10.1002/ajmg.1397.

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13

Pyle, Angela, Helen J. Nightingale, Helen Griffin, Angela Abicht, Janbernd Kirschner, Ivo Baric, Mario Cuk, et al. "Respiratory chain deficiency in nonmitochondrial disease." Neurology Genetics 1, no. 1 (April 27, 2015): e6. http://dx.doi.org/10.1212/nxg.0000000000000006.

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14

Esteitie, N., and N.-G. Larsson. "Defective assembly of the respiratory chain." Acta Paediatrica 93, no. 10 (January 2, 2007): 1268–70. http://dx.doi.org/10.1111/j.1651-2227.2004.tb02920.x.

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15

Walker, Ulrich A., Steven Collins, and Edward Byrne. "Respiratory Chain Encephalomyopathies: A Diagnostic Classification." European Neurology 36, no. 5 (1996): 260–67. http://dx.doi.org/10.1159/000117269.

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16

Eubel, Holger, Jesco Heinemeyer, Stephanie Sunderhaus, and Hans-Peter Braun. "Respiratory chain supercomplexes in plant mitochondria." Plant Physiology and Biochemistry 42, no. 12 (December 2004): 937–42. http://dx.doi.org/10.1016/j.plaphy.2004.09.010.

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17

Bott, Michael, and Axel Niebisch. "The respiratory chain of Corynebacterium glutamicum." Journal of Biotechnology 104, no. 1-3 (September 2003): 129–53. http://dx.doi.org/10.1016/s0168-1656(03)00144-5.

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18

Bindoff, L. A., and D. M. Turnbull. "8 Defects of the respiratory chain." Baillière's Clinical Endocrinology and Metabolism 4, no. 3 (September 1990): 583–619. http://dx.doi.org/10.1016/s0950-351x(05)80069-2.

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19

Larsson, Nils-Göran, and Pierre Rustin. "Animal models for respiratory chain disease." Trends in Molecular Medicine 7, no. 12 (December 2001): 578–81. http://dx.doi.org/10.1016/s1471-4914(01)02167-0.

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20

Hicks, David B., and Terry A. Krulwich. "The respiratory chain of alkaliphilic bacteria." Biochimica et Biophysica Acta (BBA) - Bioenergetics 1229, no. 3 (May 1995): 303–14. http://dx.doi.org/10.1016/0005-2728(95)00024-d.

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21

Higgins, D. S., M. Garcia-Osuna, and J. T. Greenamyre. "Neuroleptic effects on the respiratory chain." Biological Psychiatry 39, no. 7 (April 1996): 553. http://dx.doi.org/10.1016/0006-3223(96)84132-1.

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22

Rötig, A. "Genetics of mitochondrial respiratory chain deficiencies." Revue Neurologique 170, no. 5 (May 2014): 309–22. http://dx.doi.org/10.1016/j.neurol.2013.11.006.

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23

Winge, Dennis R., and Alex Tzagoloff. "Assembly of the Mitochondrial Respiratory Chain." Biochimica et Biophysica Acta (BBA) - Molecular Cell Research 1793, no. 1 (January 2009): 1. http://dx.doi.org/10.1016/j.bbamcr.2008.11.002.

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24

Slater, E. C. "Keilin, Cytochrome, and the Respiratory Chain." Journal of Biological Chemistry 278, no. 19 (January 30, 2003): 16455–61. http://dx.doi.org/10.1074/jbc.x200011200.

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25

Rustin, Pierre, Dominique Chretien, Thomas Bourgeron, Anne Wucher, Jean-Marie Saudubray, Agnes Rotig, and Arnold Munnich. "Assessment of the mitochondrial respiratory chain." Lancet 338, no. 8758 (July 1991): 60. http://dx.doi.org/10.1016/0140-6736(91)90057-v.

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26

Lincke, C., C. van den Bogert, L. Nijtmans, R. Wanders, P. Tamminga, and P. Barth. "Cerebellar Hypoplasia in Respiratory Chain Dysfunction." Neuropediatrics 27, no. 04 (August 1996): 216–18. http://dx.doi.org/10.1055/s-2007-973792.

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27

Gauthier-Villars, Marion, P. Landrieu, Valérie Cormier-Daire, E. Jacquemin, D. Chrétien, Agnès Rötig, P. Rustin, A. Munnich, and Pascale de Lonlay. "Respiratory Chain Deficiency in Alpers Syndrome." Neuropediatrics 32, no. 3 (June 2001): 150–52. http://dx.doi.org/10.1055/s-2001-16614.

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28

Brzezinski, Peter. "Regulatory Interactions within the Respiratory Chain." Biochimica et Biophysica Acta (BBA) - Bioenergetics 1859 (September 2018): e13. http://dx.doi.org/10.1016/j.bbabio.2018.09.040.

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29

Suomalainen, Anu. "Biomarkers for mitochondrial respiratory chain disorders." Journal of Inherited Metabolic Disease 34, no. 2 (October 13, 2010): 277–82. http://dx.doi.org/10.1007/s10545-010-9222-3.

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30

Mayr, Johannes A., Tobias B. Haack, Peter Freisinger, Daniela Karall, Christine Makowski, Johannes Koch, René G. Feichtinger, et al. "Spectrum of combined respiratory chain defects." Journal of Inherited Metabolic Disease 38, no. 4 (March 17, 2015): 629–40. http://dx.doi.org/10.1007/s10545-015-9831-y.

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31

Capaldi, Roderick A. "Mitochondrial myopathies and respiratory chain proteins." Trends in Biochemical Sciences 13, no. 4 (April 1988): 144–48. http://dx.doi.org/10.1016/0968-0004(88)90073-4.

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32

Wolvetang, Ernst J., Karina L. Johnson, Kenia Krauer, Steve J. Ralph, and Anthony W. Linnane. "Mitochondrial respiratory chain inhibitors induce apoptosis." FEBS Letters 339, no. 1-2 (February 14, 1994): 40–44. http://dx.doi.org/10.1016/0014-5793(94)80380-3.

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33

Kogot-Levin, Aviram, and Ann Saada. "Ceramide and the mitochondrial respiratory chain." Biochimie 100 (May 2014): 88–94. http://dx.doi.org/10.1016/j.biochi.2013.07.027.

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34

Alvarez, Cristina Uribe, Natalia Chiquete-Félix, Salvador Uribe-Carvajal, and Antonio Peña. "Exploring the Staphylococcus Epidermidis Respiratory Chain." Biophysical Journal 108, no. 2 (January 2015): 602a. http://dx.doi.org/10.1016/j.bpj.2014.11.3280.

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35

Cogliati, Sara, Christian Frezza, Maria Eugenia Soriano, Tatiana Varanita, Ruben Quintana-Cabrera, Mauro Corrado, Sara Cipolat, et al. "Mitochondrial Cristae Shape Determines Respiratory Chain Supercomplexes Assembly and Respiratory Efficiency." Cell 155, no. 1 (September 2013): 160–71. http://dx.doi.org/10.1016/j.cell.2013.08.032.

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36

Altinterim, Basar. "The Effect in respiratory chain of CoQ10." Sakarya Medical Journal 3, no. 2 (2013): 51–54. http://dx.doi.org/10.5505/sakaryamj.2013.35220.

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37

Oexle, Konrad, and Angelika Zwirner. "Advanced Telomere Shortening in Respiratory Chain Disorders." Human Molecular Genetics 6, no. 6 (June 1, 1997): 905–8. http://dx.doi.org/10.1093/hmg/6.6.905.

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38

Fernandez-Mosquera, Lorena, King Faisal Yambire, Renata Couto, Leonardo Pereyra, Kamil Pabis, Amy H. Ponsford, Cátia V. Diogo, Massimiliano Stagi, Ira Milosevic, and Nuno Raimundo. "Mitochondrial respiratory chain deficiency inhibits lysosomal hydrolysis." Autophagy 15, no. 9 (March 27, 2019): 1572–91. http://dx.doi.org/10.1080/15548627.2019.1586256.

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39

Chen, Ming, Leif P. Andersen, Lin Zhai, and Arsalan Kharazmi. "Characterization of the respiratory chain ofHelicobacter pylori." FEMS Immunology & Medical Microbiology 24, no. 2 (June 1999): 169–74. http://dx.doi.org/10.1111/j.1574-695x.1999.tb01278.x.

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40

Rich, P. R. "The molecular machinery of Keilin's respiratory chain." Biochemical Society Transactions 31, no. 6 (December 1, 2003): 1095–105. http://dx.doi.org/10.1042/bst0311095.

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Keilin's classic paper of 1925 [Keilin (1925) Proc. R. Soc. London Ser. B 100, 129–151], achieved with simple, but elegant, techniques, describes the cytochrome components of the respiratory chain and their roles in intracellular respiration and oxygen consumption. Since that time, a tremendous amount of work has clarified the intricate details of the prosthetic groups, cofactors and proteins that comprise the respiratory chain and associated machinery for ATP synthesis. The work has culminated in advanced crystallographic and spectroscopic methods that provide structural and mechanistic details of this mitochondrial molecular machinery, in many instances to atomic level. I review here the current state of understanding of the mitochondrial respiratory chain in terms of structures and dynamics of the component proteins and their roles in the biological electron and proton transfer processes that result in ATP synthesis. These advances, together with emerging evidence of further diverse roles of mitochondria in health and disease, have prompted a new era of interest in mitochondrial function.
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41

Finsterer, Josef, Georg Pelzl, Max Zartl, Elisabeth Unger, and Bruno Mamoli. "Video-EEG Monitoring in Respiratory Chain Disorders." Clinical Electroencephalography 32, no. 1 (January 2001): 24–27. http://dx.doi.org/10.1177/155005940103200107.

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42

Turrens, Julio F. "Superoxide Production by the Mitochondrial Respiratory Chain." Bioscience Reports 17, no. 1 (February 1, 1997): 3–8. http://dx.doi.org/10.1023/a:1027374931887.

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This mini-review describes the role of different mitochondrial components in the formation of reactive oxygen species under normal and pathological conditions and the effect of inhibitors and uncouplers on superoxide formation.
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43

Cholley, Florence, P. Edery, D. Ricquier, S. Peudenier, A. Slama, and M. Tardieu. "Mitochondrial Respiratory Chain Deficiency Revealed by Hypothermia." Neuropediatrics 32, no. 2 (April 2001): 104–6. http://dx.doi.org/10.1055/s-2001-13878.

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44

Rötig, Agnès. "Genetic bases of mitochondrial respiratory chain disorders." Diabetes & Metabolism 36, no. 2 (April 2010): 97–107. http://dx.doi.org/10.1016/j.diabet.2009.11.002.

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45

Castro-Gago, M., M. O. Blanco-Barca, and J. Eirís-Puñal. "Epilepsy and Respiratory Chain Defects in Children." Neuropediatrics 40, no. 03 (August 2009): 152. http://dx.doi.org/10.1055/s-0029-1243186.

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46

Morris, Andrew A. M. "Mitochondrial respiratory chain disorders and the liver." Liver International 19, no. 5 (October 1999): 357–68. http://dx.doi.org/10.1111/j.1478-3231.1999.tb00063.x.

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47

Schäfer, Eva, Holger Seelert, Nicole H. Reifschneider, Frank Krause, Norbert A. Dencher, and Janet Vonck. "Architecture of Active Mammalian Respiratory Chain Supercomplexes." Journal of Biological Chemistry 281, no. 22 (March 20, 2006): 15370–75. http://dx.doi.org/10.1074/jbc.m513525200.

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48

Piemonte, F., C. Casali, R. Carrozzo, H. Schägger, C. Patrono, A. Tessa, G. Tozzi, et al. "Respiratory chain defects in hereditary spastic paraplegias." Neuromuscular Disorders 11, no. 6-7 (September 2001): 565–69. http://dx.doi.org/10.1016/s0960-8966(01)00214-0.

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49

von Kleist-Retzow, Jürgen-Christoph, Valérie Cormier-Daire, Géraldine Viot, Alice Goldenberg, Becky Mardach, Jeanne Amiel, Philippe Saada, et al. "Antenatal manifestations of mitochondrial respiratory chain deficiency." Journal of Pediatrics 143, no. 2 (August 2003): 208–12. http://dx.doi.org/10.1067/s0022-3476(03)00130-6.

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50

Lobo-Jarne, Teresa, and Cristina Ugalde. "Respiratory chain supercomplexes: Structures, function and biogenesis." Seminars in Cell & Developmental Biology 76 (April 2018): 179–90. http://dx.doi.org/10.1016/j.semcdb.2017.07.021.

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