Mitochondrial Oxidative Phosphorylation

1. Front Matter

   Pages i-xi

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2. Introduction to Mitochondrial Oxidative Phosphorylation

   • Bernhard Kadenbach

   Pages 1-11

3. Mitochondrial Dynamics: The Intersection of Form and Function

   • Andrew Ferree, Orian Shirihai

   Pages 13-40

4. Biogenesis of Mitochondrial Proteins

   • Johannes M. Herrmann, Sebastian Longen, Daniel Weckbecker, Matthieu Depuydt

   Pages 41-64

5. Assembly Factors of Human Mitochondrial Respiratory Chain Complexes: Physiology and Pathophysiology

   • Daniele Ghezzi, Massimo Zeviani

   Pages 65-106

6. Supramolecular Organisation of the Mitochondrial Respiratory Chain: A New Challenge for the Mechanism and Control of Oxidative Phosphorylation

   • Giorgio Lenaz, Maria Luisa Genova

   Pages 107-144

7. Molecular Mechanisms of Superoxide Production by the Mitochondrial Respiratory Chain

   • Stefan Dröse, Ulrich Brandt

   Pages 145-169

8. Studies on the Function and Regulation of Mitochondrial Uncoupling Proteins

   • Richard K. Porter

   Pages 171-184

9. Evolution of the Couple Cytochrome c and Cytochrome c Oxidase in Primates

   • Denis Pierron, Derek E. Wildman, Maik Hüttemann, Thierry Letellier, Lawrence I. Grossman

   Pages 185-213

10. Reaction Mechanism of Mammalian Mitochondrial Cytochrome c Oxidase

    • Shinya Yoshikawa, Kazumasa Muramoto, Kyoko Shinzawa-Itoh

    Pages 215-236

11. Phosphorylation of Mammalian Cytochrome c and Cytochrome c Oxidase in the Regulation of Cell Destiny: Respiration, Apoptosis, and Human Disease

    • Maik Hüttemann, Icksoo Lee, Lawrence I. Grossman, Jeffrey W. Doan, Thomas H. Sanderson

    Pages 237-264

12. Individual Biochemical Behaviour Versus Biological Robustness: Spotlight on the Regulation of Cytochrome c Oxidase

    • Rabia Ramzan, Petra Weber, Bernhard Kadenbach, Sebastian Vogt

    Pages 265-281

13. Bigenomic Regulation of Cytochrome c Oxidase in Neurons and the Tight Coupling Between Neuronal Activity and Energy Metabolism

    • Margaret T. T. Wong-Riley

    Pages 283-304

14. Cytochrome c Oxidase and Its Role in Neurodegeneration and Neuroprotection

    • Susanne Arnold

    Pages 305-339

15. The Many Clinical Faces of Cytochrome c Oxidase Deficiency

    • Salvatore DiMauro, Kurenai Tanji, Eric A. Schon

    Pages 341-357

16. Back Matter

    Pages 359-372

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This book will describe the nuclear encoded genes and their expressed proteins of mitochondrial oxidative phosphorylation. Most of these genes occur in eukaryotic cells, but not in bacteria or archaea. The main function of mitochondria, the synthesis of ATP, is performed at subunits of proton pumps (complexes I, III, IV and V), which are encoded on mitochondrial DNA. The nuclear encoded subunits have mostly a regulatory function. However, the specific physiological functions of the nuclear encoded subunits of complexes I, III, IV, and V are mostly unknown. New data indicates that they are essential for life of higher organisms, which is characterized by an adult life without cell division (postmeiotic stage) in most tissues, after the juvenile growth. For complex IV (cytochrome c oxidase) some of these subunits occur in tissue-specific (subunits IV, VIa, VIb, VIIa, VIII), developmental-specific (subunits IV, VIa, and VIIa) as well as species-specific isoforms. Defective genes of some subunits were shown to induce mitochondrial diseases. Mitochondrial genes and human diseases will also be covered.

• gene expression

• Philipps-Universitaet Marburg, Marburg, Germany

  Bernhard Kadenbach

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