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1.5 Electron Transport. Structure, Redox-Coupled Protonmotive Activity, and Pathological Disorders of Respiratory Chain Complexes

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Handbook of Neurochemistry and Molecular Neurobiology

Abstract:

This chapter is intended to provide an overview of mitochondrial respiratory chain complexes from protein structure and functional mechanisms to their biogenesis and genetic disorders in neurological and other diseases. The general features and the electron transfer centers of the protonmotive respiratory chain is first dealt with. This section is followed by a description of the protein structure of the four redox complexes of the chain. A section is devoted to mechanism of the proton pump of complexes I, III and IV with particular emphasis to complex IV. The last two sections cover aspects of the biogenesis of the redox complexes and their genetic disorders in human pathology respectively.

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Acknowledgments

This work was supported by grants from the National Project on Bioenergetics: functional genetics, functional mechanisms, and physiopathological aspects, 2003, MIUR, Italy, and the Center of Excellence on Comparative Genomics, University of Bari.

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  1. S. Papa
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  2. V. Petruzzella
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  3. S. Scacco
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Editors and Affiliations

  1. Center for Neurochemistry, Nathan S. Kline Institute for Psychiatric Research, 140 Old Orangeburg Road, 10962, Orangeburg, Newyork, USA

    Abel Lajtha (Director) (Director)

  2. Weill Medical College of Cornell University, Burke Medical Research Institute, 785 Mamaroneck Avenue, White Plains, NY, 10605, USA

    Gary E. Gibson Ph.D. (Professor of Neuroscience Department of Neurology and Neuroscience) (Professor of Neuroscience Department of Neurology and Neuroscience)

  3. Dept. Neurology and Physiology & Biophysics, University Arkansas for Medical Sciences, Shorey Bldg. Room 715, Mail Slot 830 4301 W. Markham St., Little Rock, AR, 72205, USA

    Gerald A. Dienel Ph.D. (Professor) (Professor)

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Papa, S., Petruzzella, V., Scacco, S. (2007). 1.5 Electron Transport. Structure, Redox-Coupled Protonmotive Activity, and Pathological Disorders of Respiratory Chain Complexes. In: Lajtha, A., Gibson, G.E., Dienel, G.A. (eds) Handbook of Neurochemistry and Molecular Neurobiology. Springer, Boston, MA. https://doi.org/10.1007/978-0-387-30411-3_5

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