Mitochondrial Oxidative Phosphorylation: Nuclear-Encoded Genes, Enzyme Regulation, and Pathophysiology

✍ Scribed by Bernhard Kadenbach (auth.), Bernhard Kadenbach (eds.)

• Publisher: Springer-Verlag New York
• Year: 2012
• Tongue: English
• Leaves: 377
• Series: Advances in Experimental Medicine and Biology 748
• Edition: 1
• Category: Library

✦ Synopsis

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.

✦ Table of Contents

Front Matter....Pages i-xi
Introduction to Mitochondrial Oxidative Phosphorylation....Pages 1-11
Mitochondrial Dynamics: The Intersection of Form and Function....Pages 13-40
Biogenesis of Mitochondrial Proteins....Pages 41-64
Assembly Factors of Human Mitochondrial Respiratory Chain Complexes: Physiology and Pathophysiology....Pages 65-106
Supramolecular Organisation of the Mitochondrial Respiratory Chain: A New Challenge for the Mechanism and Control of Oxidative Phosphorylation....Pages 107-144
Molecular Mechanisms of Superoxide Production by the Mitochondrial Respiratory Chain....Pages 145-169
Studies on the Function and Regulation of Mitochondrial Uncoupling Proteins....Pages 171-184
Evolution of the Couple Cytochrome c and Cytochrome c Oxidase in Primates....Pages 185-213
Reaction Mechanism of Mammalian Mitochondrial Cytochrome c Oxidase....Pages 215-236
Phosphorylation of Mammalian Cytochrome c and Cytochrome c Oxidase in the Regulation of Cell Destiny: Respiration, Apoptosis, and Human Disease....Pages 237-264
Individual Biochemical Behaviour Versus Biological Robustness: Spotlight on the Regulation of Cytochrome c Oxidase....Pages 265-281
Bigenomic Regulation of Cytochrome c Oxidase in Neurons and the Tight Coupling Between Neuronal Activity and Energy Metabolism....Pages 283-304
Cytochrome c Oxidase and Its Role in Neurodegeneration and Neuroprotection....Pages 305-339
The Many Clinical Faces of Cytochrome c Oxidase Deficiency....Pages 341-357
Back Matter....Pages 359-372

✦ Subjects

Human Genetics; Gene Expression; Cell Biology