Chapter 18

Where to Start

Guarente, L. 2008. Mitochondria: A nexus for aging, calorie restriction, and sirtuins? Cell 132:171–176.

Wallace, D. C. 2007. Why do we still have a maternally inherited mitochondrial DNA? Insights from evolutionary medicine. Annu. Rev. Biochem. 76:781–821.

Hosler, J. P., Ferguson-Miller, S., and Mills, D. A. 2006. Energy transduction: Proton transfer through the respiratory complexes. Annu. Rev. Biochem. 75:165–187.

Gray, M. W., Burger, G., and Lang, B. F. 1999. Mitochondrial evolution. Science 283:1476–1481.

Shultz, B. E., and Chan, S. I. 2001. Structures and proton-pumping strategies of mitochondrial respiratory enzymes. Annu. Rev. Biophys. Biomol. Struct. 30:23–65.

Books

Scheffler, I. E. 2007. Mitochondria. Wiley.

Lane, N. 2005. Power, Sex, Suicide: Mitochondria and the Meaning of Life. Oxford.

Nicholls, D. G., and Ferguson, S. J. 2013. Bioenergetics (4th ed.). Academic Press.

B19

Electron-Transport Chain

Baradaran, R., Berrisford, J. M., Minhas, G. S., and Sazanov, L. A. 2013. Crystal structure of the entire respiratory complex I. Nature 494:443–448.

Lapuente-Brun, E., Moreno-Loshuertos, R., Acín-Pérez, R., Latorre-Pellicer, A., Colás, C., Balsa, E., Perales-Clemente, E., Quirós, P. M., Calvo, E., Rodríguez-Hernández, M. A., et al. 2013. Supercomplex assembly determines electron flux in the mitochondrial electron transport chain. Science 340:1567–1570.

Cammack, R. 2012. Iron-sulfur proteins. The Biochemist 35:14–17.

Yoshikawa, S., Muramoto, K., and Shinzawa-Itoh, K. 2011. Proton-pumping mechanism of cytochrome c oxidase. Annu. Rev. Biophys. 40:205–23.

Qin, L., Liu, J., Mills, D. A., Proshlyakov, D. A., Hiser, C., and Ferguson-Miller, S. 2009. Redox-dependent conformational changes in cytochrome c oxidase suggest a gating mechanism for proton uptake. Biochemistry 48:5121–5130.

Lill, R. 2009. Function and biogenesis of iron–sulphur proteins. Nature 460:831–838.

Cooley, C. W., Lee, D.-W., and Daldal, F. 2009. Across membrane communication between the Qo and Qi active sites of cytochrome bc1. Biochemistry 48:1888–1899.

Verkhovskaya, M. L., Belevich, N., Euro, L., Wikström, M., and. Verkhovsky, M. I. 2008. Real-time electron transfer in respiratory complex I. Proc. Natl. Acad. Sci. U.S.A. 105:3763–3767.

Acín-Pérez, R., Fernández-Silva, P., Peleato, M. L., Pérez-Martos, A., and Enriquez, J. A. 2008. Respiratory active mitochondrial supercomplexes. Mol. Cell 32:529–539.

Kruse, S. E., Watt, W. C., Marcinek, D. J., Kapur, R. P., Schenkman, K. A., and Palmiter, R. D. 2008. Mice with mitochondrial Complex I deficiency develop a fatal encephalomyopathy. Cell Metab. 7:312–320.

Sun, F., Huo, X., Zhai, Y., Wang, A., Xu, J., Su, D., Bartlam, M., and Ral, Z. 2005. Crystal structure of mitochondrial respiratory membrane protein complex II. Cell 121:1043–1057.

Crofts, A. R. 2004. The cytochrome bc1 complex: Function in the context of structure. Annu. Rev. Physiol. 66:689–733.

Bianchi, C., Genova, M. L., Castelli, G. P., and Lenaz, G. 2004. The mitochondrial respiratory chain is partially organized in a supramolecular complex. J. Biol. Chem. 279:36562–36569.

Cecchini, G. 2003. Function and structure of Complex II of the respiratory chain. Annu. Rev. Biochem. 72:77–109.

Lange, C., and Hunte, C. 2002. Crystal structure of the yeast cytochrome bc1 complex with its bound substrate cytochrome c. Proc. Natl. Acad. Sci. U.S.A. 99:2800–2805.

ATP Synthase

Toei, M., and Noji, H. 2013. Single-molecule analysis of F0F1-ATP synthase inhibited by N, N-dicyclohexylcarbodiimide. J. Biol. Chem. 288:25717–25726.

Watt, I. N., Montgomery, M. G., Runswick, M. J., Leslie, A. G. W., and Walker, J. E. 2010. Bioenergetic cost of making an adenosine triphosphate molecule in animal mitochondria. Proc. Natl. Acad. Sci. U.S.A. 107:16823–16827.

Wittig, I., and Hermann, S. 2009. Supramolecular organization of ATP synthase and respiratory chain in mitochondrial membranes. Biochim. Biophys. Acta 1787:672–680.

Junge, W., Sielaff, H., and Engelbrecht S. 2009. Torque generation and elastic power transmission in the rotary F0F1-ATPase. Nature 459:364–370.

von Ballmoos, C., Cook, G. M., and Dimroth, P. 2008. Unique rotary ATP synthase and its biological diversity. Annu. Rev. Biophys. 37:43–64.

Adachi, K., Oiwa, K., Nishizaka, T., Furuike, S., Noji, H., Itoh, H., Yoshida, M., and Kinosita, K., Jr. 2007. Coupling of rotation and catalysis in F1-ATPase revealed by single-molecule imaging and manipulation. Cell 130:309–321.

Chen, C., Ko, Y., Delannoy, M., Ludtke, S. J., Chiu, W., and Pedersen, P. L. 2004. Mitochondrial ATP synthasome: Three-dimensional structure by electron microscopy of the ATP synthase in complex formation with the carriers for Pi and ADP/ATP. J. Biol. Chem. 279:31761–31768.

Noji, H., and Yoshida, M. 2001. The rotary machine in the cell: ATP synthase. J. Biol. Chem. 276:1665–1668.

Yasuda, R., Noji, H., Kinosita, K., Jr., and Yoshida, M. 1998. F1-ATPase is a highly efficient molecular motor that rotates with discrete 120 degree steps. Cell 93:1117–1124.

Noji, H., Yasuda, R., Yoshida, M., and Kinosita, K., Jr., 1997. Direct observation of the rotation of F1-ATPase. Nature 386:299–302.

Tsunoda, S. P., Aggeler, R., Yoshida, M., and Capaldi, R. A. 2001. Rotation of the c subunit oligomer in fully functional F1 F0 ATP synthase. Proc. Natl. Acad. Sci. U.S.A. 987:898–902.

Gibbons, C., Montgomery, M. G., Leslie, A. G. W., and Walker, J. 2000. The structure of the central stalk in F1-ATPase at 2.4 Å resolution. Nat. Struct. Biol. 7:1055–1061.

Sambongi, Y., Iko, Y., Tanabe, M., Omote, H., Iwamoto-Kihara, A., Ueda, I., Yanagida, T., Wada, Y., and Futai, M. 1999. Mechanical rotation of the c subunit oligomer in ATP synthase (F0F1): Direct observation. Science 286:1722–1724.

Translocators and Channels

Villarroya, F., and Vidal-Puig, A. 2013. Beyond the sympathetic tone: The new brown fat activators. Cell Metab.17:638–643.

Rey, M., Forest, E., and Pelosi, L. 2012. Exploring the conformational dynamics of the bovine ADP/ATP carrier in mitochondria. Biochemistry 51:9727–9735.

Divakaruni, A. S., Humphrey, D. M., and Brand, M. D. 2012. Fatty acids change the conformation of uncoupling protein 1 (UCP1). J. Biol. Chem. 44:36845–36853.

Fedorenko, A., Lishko, P. V., and Kirichok, Y. 2012. Mechanism of fatty-acid-dependent UCP1 uncoupling in brown fat mitochondria. Cell 151:400–413.

van Marken Lichtenbelt, W. D., Vanhommerig, J. W., Smulders, N. M., Drossaerts, J. M., Kemerink, G. J., Bouvy, N. D., Schrauwen, P., and Teule, G. J. 2009. Cold-activated brown adipose tissue in healthy men. New Engl. J. Med. 360:1500–1508.

Cypess, A. M., Sanaz Lehman, S., Gethin Williams, G., Tal, I., Rodman, D., Goldfine, A. B., Kuo, F. C., Palmer, E. L., Tseng, Y.-H., Doria, A., et al. 2009. Identification and importance of brown adipose tissue in adult humans. New Engl. J. Med. 360:1509–1517.

Virtanen, K. A., Lidell, M. E., Orava, J., Heglind, M., Westergren, R., Niemi, T., Taittonen, M., Laine, J., Savisto, N.-J., Enerbäck, S., et al. 2009. Functional brown adipose tissue in healthy adults. New Engl. J. Med. 360:1518–1525.

Bayrhuber, M., Meins, T., Habeck, M., Becker, S., Giller, K., Villinger, S., Vonrhein, C., Griesinger, C., Zweckstetter, M., and Zeth, K. 2008. Structure of the human voltage-dependent anion channel. Proc. Natl. Acad. Sci. U.S.A. 105:15370–15375.

Bamber, L., Harding, M., Monné, M., Slotboom, D.-J., and Kunji, E. R. 2007. The yeast mitochondrial ADP/ATP carrier functions as a monomer in mitochondrial membranes. Proc. Natl. Acad. Sci. U.S.A. 10:10830–10843.

Pebay-Peyroula, E., Dahout, C., Kahn, R., Trézéguet, V., Lauquin, G. J.-M., and Brandolin, G. 2003. Structure of mitochondrial ADP/ATP carrier in complex with carboxyatractyloside. Nature 246:39–44.

Reactive Oxygen Species, Superoxide Dismutase, and Catalase

Sena, L. A., and Chandel, N. S. 2012. Physiological roles of mitochondrial reactive oxygen species. Mol. Cell 48:158–167.

Forman, H. J., Maiorino, M., and Ursini, F. 2010. Signaling functions of reactive oxygen species. Biochemistry 49:835–842.

Murphy, M. P. 2009. How mitochondria produce reactive oxygen species. Biochem. J. 417:1–13.

B20

Leitch, J. M., Yick, P. J., and Culotta, V. V. 2009. The right to choose: Multiple pathways for activating copper, zinc superoxide dismutase. J. Biol. Chem. 284:24679–24683.

Winterbourn, C. C. 2008. Reconciling the chemistry and biology of reactive oxygen species. Nat. Chem. Biol. 4:278–286.

Veal, E. A., Day, A. M., and Morgan, B. A. 2007. Hydrogen peroxide sensing and signaling. Mol. Cell 26:1–14.

Stone, J. R., and Yang, S. 2006. Hydrogen peroxide: A signaling messenger. Antioxid. Redox Signal. 8:243–270.

Valentine, J. S., Doucette, P. A., and Potter S. Z. 2005. Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis. Annu. Rev. Biochem. 74:563–593.

Mitochondrial Diseases

Papa, S., and De Rasmo, D. 2013. Complex I deficiencies in neurological disorders. Trends Mol. Med. 19:61–69.

Koopman, W. J. H., Willems, P. H. G. M., and Smeitink, J. A. M. 2012. Monogenic mitochondrial disorders. New Engl. J. Med. 366:1132–41.

Lina, C. S., Sharpley, M. S., Fan W., Waymire, K. G., Sadun, A. A., Carelli, V., Ross-Cisneros, F. N., Baciu, P., Sung, E., McManus, M. J., et al. 2012. Mouse mtDNA mutant model of Leber hereditary optic neuropathy. Proc. Natl. Acad. Sci. U.S.A. 109:20065–20070.

Mitochondria Disease. 2009. A compendium of nine articles on mitochondrial diseases. Biochem. Biophys. Acta Mol. Basis Disease 1792:1095–1167.

Cicchetti, F., Drouin-Ouellet, J., and Gross, R. E. 2009. Environmental toxins and Parkinson’s disease: What have we learned from pesticide-induced animal models? Trends Pharm. Sci. 30:475–483.

DiMauro, S., and Schon, E. A. 2003. Mitochondrial respiratory-chain disease. New Engl. J. Med. 348:2656–2668.

Smeitink, J., van den Heuvel, L., and DiMauro, S. 2001. The genetics and pathology of oxidative phosphorylation. Nat. Rev. Genet. 2:342–352.

Apoptosis

Qi, S., Pang, Y., Hu, Q., Liu, Q., Li, H., Zhou, Y., He, T., Liang, Q., Liu, Y., Yuan, X., et al. 2010. Crystal structure of the Caenorhabditis elegans apoptosome reveals an octameric assembly of CED-4. Cell 141:446–457.

Chan, D. C. 2006. Mitochondria: Dynamic organelles in disease, aging, and development. Cell 125:1241–1252.

Green, D. R. 2005. Apoptotic pathways: Ten minutes to dead. Cell 121:671–674.

Historical Aspects

Prebble, J., and Weber, B. 2003. Wandering in the Gardens of the Mind: Peter Mitchell and the Making of Glynn. Oxford.

Mitchell, P. 1979. Keilin’s respiratory chain concept and its chemiosmotic consequences. Science 206:1148–1159.

Preeble, J. 2002. Peter Mitchell and the ox phos wars. Trends Biochem. Sci. 27:209–212.

Mitchell, P. 1976. Vectorial chemistry and the molecular mechanics of chemiosmotic coupling: Power transmission by proticity. Biochem. Soc. Trans. 4:399–430.

Racker, E. 1980. From Pasteur to Mitchell: A hundred years of bioenergetics. Fed. Proc. 39:210–215.

Kalckar, H. M. 1991. Fifty years of biological research: From oxidative phosphorylation to energy requiring transport and regulation. Annu. Rev. Biochem. 60:1–37.