Note: Intro -- MITOCHONDRIA -- CONTENTS -- Preface -- 1 History -- References -- 2 Evolutionary Origin of Mitochondria -- References -- 3 Structure and Morphology: Integration into the cell -- 3.1 Structure and Morphology -- 3.2 Integration Into the Cell -- 3.2.1 Distribution in the Cytosol -- 3.2.2 Interaction With Cytoskeleton -- 3.2.3 Mitochondrial Shape Changes -- 3.2.4 Distribution During Cell Division -- 3.2.5 Behavior During Cell Differentiation -- 3.2.6 Turnover and Degradation -- 3.2.7 Unsolved Problems for the Future -- References -- 4 Biogenesis -- 4.1 The Mitochondrial Genome -- 4.1.1 Introduction -- 4.1.2 The Mitochondrial Genome in Metazoans -- 4.1.3 The Mitochondrial Genome in Plants -- 4.1.4 The Mitochondrial Genome in Fungi -- 4.1.5 The Mitochondrial Genome in Kinetoplastid Protozoa -- 4.1.6 Mitochondrial Plasmids -- 4.2 Nuclear Genes Encoding Mitochondrial Proteins -- 4.2.1 Enzymes Required for Mitochondrial Gene Expression -- 4.2.2 Nucleomitochondrial Interactions -- 4.3 Replication of Mitochondrial DNA -- 4.4 Transcription of mtDNA-RNA Metabolism -- 4.4.1 Transcription in Mammalian Mitochondria -- 4.4.2 Transcription of mtDNA in the Yeast Saccharomyces cerevisiae -- 4.4.3 Transcription of mtDNA in Plant Mitochondria -- 4.4.4 Transcriptional Termination -- 4.4.5 RNA Processing in Mitochondria -- 4.4.6 RNA Editing in Kinetoplastic Protozoa -- 4.4.7 RNA Editing in Plant Mitochondria -- 4.4.8 Control of mRNA Levels by Turnover -- 4.5 Translation of Mitochondrial mRNAs -- 4.5.1 Introduction -- 4.5.2 Codon Usage and tRNA Structure -- 4.5.3 Mitochondrial Ribosomes -- 4.5.4 cis-Acting Elements of Mitochondrial mRNA -- 4.5.5 Translation Factors -- 4.6 Protein Import Into Mitochondria -- 4.7 Import of tRNA Into Mitochondria -- 4.8 Regulated Protein Degradation in Mitochondria -- 4.9 Unsolved Problems -- References. , 5 Mitochondrial Electron Transport and Oxidative Phosphorylation -- 5.1 Historical Introduction -- 5.2 The Electron Transport Chain -- 5.2.1 Biochemical Components -- 5.2.2 Physical Separation of the Complexes of the Electron Transport Chain -- 5.3 Electron Transport in Other Organisms -- 5.3.1 NAD(P)H Dehydrogenases -- 5.3.2 A Cyanide-Insensitive Electron Pathway -- 5.3.3 NADH Oxidation in Yeast -- 5.3.4 Energy Metabolism and NADH Oxidation in Trypanosomes -- 5.4 The Chemiosmotic Hypothesis -- 5.4.1 The Mitchell Hypothesis -- 5.4.2 The Q Cycle -- 5.4.3 Probing the Mitochondrial Membrane Potential With Fluorescent Dyes -- 5.5 ATP Synthase (F(1)F(0)-ATPase) -- 5.5.1 Introduction -- 5.5.2 X-ray Structure -- 5.5.3 ATP Synthesis and Catalytic Mechanisms -- 5.5.4 The F(0) Subcomplex and Proton Flow -- 5.6 Control of Respiration and Oxidative Phosphorylation -- 5.6.1 General Considerations -- 5.6.2 The Uncoupling Proteins in Warm-Blooded Animals -- 5.6.3 Uncoupling in Other Organisms -- 5.6.4 Transport of Small Solutes Into and Out of Mitochondria -- 5.7 Reactive Oxygen Species -- 5.8 The Role of Specific Lipids -- References -- 6 Metabolic Pathways Inside Mitochondria -- 6.1 Introduction -- 6.2 The Krebs Cycle -- 6.3 Fatty Acid Metabolism -- 6.4 The Urea Cycle -- 6.5 Biosynthesis of Heme -- 6.6 Cardiolipin and Lipid Biosynthesis/Metabolism -- 6.7 Biosynthesis of Ubiquinol (Coenzyme Q) -- References -- 7 Mitochondrial Mutations and Disease -- 7.1 In Cell Culture -- 7.1.1 Mitochondrial Mutations in Microorganisms -- 7.1.2 Respiration-Deficient Mammalian Cells -- 7.2 Molecular Genetics of Mitochondrial Diseases -- 7.2.1 Introduction -- 7.2.2 Maternal vs Sporadic Inheritance -- 7.2.3 Mapping mtDNA Deletions/Rearrangements -- 7.2.4 Maternal Inheritance of mtDNA Point Mutations -- 7.2.5 Mitochondria and Oogenesis -- 7.2.6 Clinical Aspects of mtDNA Mutations. , 7.2.7 Conclusion -- 7.3 Mitochondrial DNA and Aging -- 7.3.1 Introduction -- 7.3.2 Accumulation of mtDNA Damage and Normal Aging -- 7.3.3 Parkinson's Disease -- 7.3.4 Alzheimer's Disease -- 7.3.5 Huntington's Disease -- 7.4 Mitochondria and Apoptosis -- 7.4.1 Introduction -- 7.4.2 The Bcl-2 Protein and the Permeability Transition -- 7.4.3 Apoptosis and Cytochrome c -- 7.4.4 Protein-Protein Interactions -- 7.5 Fungal Senescence -- 7.6 Cytoplasmic Male Sterility in Plants -- References -- 8 Mitochondrial DNA Polymorphisms and Anthropology -- 8.1 Introduction -- 8.2 Human Evolution -- 8.3 Primate Evolution -- 8.4 Human Y Chromosome Variation -- 8.5 Forensic Applications -- 8.6 Future Challenges -- References -- 9 Mitochondria and Pharmacology -- 9.1 Introduction -- References -- Glossary -- Index.

Note: Intro -- MITOCHONDRIA -- CONTENTS -- Preface -- Preface to First Edition -- 1 History -- References -- 2 Evolutionary Origin of Mitochondria -- References -- 3 Structure and Morphology. Integration into the Cell -- 3.1 Structure and Morphology -- 3.2 Integration into the Cell -- 3.2.1 Distribution in the Cytosol -- 3.2.2 Interaction with Cytoskeleton -- 3.3 The Dynamics of Mitochondrial Morphology -- 3.3.1 Mitochondrial Shape Changes -- 3.3.1.1 Fission -- 3.3.1.2 Fusion -- 3.3.2 Distribution During Cell Division -- 3.3.3 During Cell Differentiation -- 3.3.4 Turnover and Degradation -- 3.3.5 Mitochondrial Alterations in Apoptosis -- 3.3.6 Unsolved Problems for the Future -- References -- 4 Biogenesis of Mitochondria -- 4.1 The Mitochondrial Genome -- 4.1.1 Introduction -- 4.1.2 The Mitochondrial Genome in Metazoans -- 4.1.3 The Mitochondrial Genome in Plants -- 4.1.4 The Mitochondrial Genome in Fungi -- 4.1.5 The Mitochondrial Genome in Kinetoplastid Protozoa -- 4.1.6 Mitochondrial Plasmids -- 4.1.6.1 Fungal Senescence -- 4.1.6.2 Phytopathogenicity -- 4.1.6.3 Cytoplasmic Male Sterility (CMS) -- 4.2 Nuclear Genes Encoding Mitochondrial Proteins -- 4.2.1 Enzymes Required for Maintenance and Expression of the Mitochondrial Genome -- 4.2.2 Nucleo-mitochondrial Interactions -- 4.2.2.1 Introduction -- 4.2.2.2 In Yeast, Saccharomyces cerevisiae -- 4.2.2.3 Regulation of Nuclear Respiratory Genes in Mammalian Cells -- 4.2.2.4 Co-evolution of Nuclear and Mitochondrial Genomes -- 4.3 Replication and Maintenance of Mitochondrial DNA -- 4.3.1 DNA Replication in Mammalian Mitochondria -- 4.3.2 mtDNA Repair in Mammalian Mitochondria -- 4.3.3 Recombination in Mammalian Mitochondria -- 4.3.4 mtDNA Maintenance and Replication in Other Organisms -- 4.4 Transcription of Mitochondrial DNA-RNA Metabolism -- 4.4.1 Transcription in Mammalian Mitochondria. , 4.4.2 Transcription of mtDNA in the Yeast Saccharomyces cerevisiae -- 4.4.3 Transcription of mtDNA in Plant Mitochondria -- 4.4.4 Transcriptional Termination -- 4.4.5 RNA Processing in Mitochondria -- 4.4.6 RNA Editing in Kinetoplastid Protozoa -- 4.4.7 Editing in Plant Mitochondria -- 4.4.8 Control of mRNA Levels by Turnover -- 4.5 Translation of Mitochondrial mRNAs -- 4.5.1 Introduction -- 4.5.2 Codon Usage and tRNA Structure -- 4.5.3 Mitochondrial Ribosomes -- 4.5.4 Cis-Acting Elements -- 4.5.5 Translation Factors -- 4.6 Protein Import into Mitochondria -- 4.6.1 Mitochondrial Targeting of Proteins -- 4.6.2 The Protein Import Machinery of Mitochondria -- 4.7 Import of Transfer RNA into Mitochondria -- 4.8 Regulated Protein Degradation in Mitochondria -- References -- 5 Mitochondrial Electron Transfer and Oxidative Phosphorylation -- 5.1 Historical Introduction -- 5.2 The Electron Transport Chain -- 5.2.1 The Biochemical Components -- 5.2.2 Physical Separation of the Complexes of the ETC -- 5.2.2.1 Biochemical Fractionations -- 5.2.2.2 Supercomplexes -- 5.2.3 Introduction to Bioenergetics -- 5.2.4 Complex I -- 5.2.5 Complex II -- 5.2.5.1 Nuclear Versus Mitochondrial Location of Complex II Genes -- 5.2.6 Complex III -- 5.2.7 Complex IV -- 5.2.8 The Assembly of the Electron Transport Chain Complexes -- 5.3 Electron Transport in Other Organisms -- 5.3.1 NAD(P)H Dehydrogenases -- 5.3.2 A Cyanide-Insensitive Electron Pathway -- 5.3.3 NADH Oxidation in Yeasts -- 5.3.4 Energy Metabolism and NADH Oxidation in Trypanosomes -- 5.4 The Chemiosmotic Hypothesis -- 5.4.1 The Mitchell Hypothesis -- 5.4.2 The Q Cycle -- 5.4.3 Probing the Mitochondrial Membrane Potential with Fluorescent Dyes -- 5.5 ATP Synthase (F(1)F(0)-ATPase) -- 5.5.1 Introduction -- 5.5.2 X-Ray Structure -- 5.5.3 ATP Synthesis and Catalytic Mechanisms. , 5.5.4 The F(0) Subcomplex and Proton Flow -- 5.5.5 Assembly of Complex V and Dimerization -- 5.6 Control of Respiration and Oxidative Phosphorylation -- 5.6.1 General Considerations -- 5.6.1.1 The Role of Substrates -- 5.6.2 The Uncoupling Proteins in Warm-Blooded Animals -- 5.6.3 Uncoupling in Other Organisms -- 5.6.3.1 In Saccharomyces cerevisiae -- 5.6.3.2 In Plants -- 5.7 Reactive Oxygen Species -- 5.8 Nitric Oxide (NO) -- 5.9 The Role of Specific Lipids -- References -- 6 Metabolic Pathways Inside Mitochondria -- 6.1 Introduction -- 6.2 The Krebs Cycle -- 6.3 Fatty Acid Metabolism -- 6.4 The Urea Cycle -- 6.5 Biosynthesis of Heme -- 6.6 Cardiolipin and Lipid Biosynthesis/Metabolism -- 6.7 Biosynthesis of Ubiquinol (Coenzyme Q) -- 6.8 Biosynthesis of Fe-S Centers -- 6.9 Transport of Small Solutes into and out of Mitochondria -- 6.9.1 Introduction -- 6.9.2 Porin Alias VDAC -- 6.9.3 The ADP/ATP Translocator -- 6.9.4 The Mitochondrial Carrier Protein Family -- 6.9.5 Cation Transport -- 6.9.5.1 Transport of Calcium and Its Physiological Role -- 6.9.6 The Mitochondrial Permeability Transition -- References -- 7 Mitochondrial Mutations and Disease -- 7.1 General Introduction -- 7.2 In Cell Culture -- 7.2.1 Mitochondrial Mutations in Microorganisms -- 7.2.2 Mitochondrial Mutations in Mammalian Cells in Culture -- 7.3 Molecular Genetics of Human Mitochondrial Diseases -- 7.3.1 Introduction -- 7.3.2 Maternal Versus Sporadic Inheritance -- 7.3.3 Mapping mtDNA Deletions/Rearrangements -- 7.3.4 mtDNA Point Mutations and Maternal Inheritance -- 7.3.5 Mitochondria and Oogenesis -- 7.3.6 Clinical Aspects of Mitochondrial DNA Mutations -- 7.3.6.1 MtDNA Deletions: Kearns-Sayre Syndrome and Pearson Syndrome -- 7.3.6.2 Familial Mitochondrial DNA Depletion -- 7.3.6.3 Point Mutations -- 7.3.7 Nuclear Mutations and Mitochondrial Disease. , 7.3.7.1 Defective Electron Transport Chain -- 7.3.7.2 MtDNA Maintenance and Replication -- 7.3.7.3 Friedreich's Ataxia -- 7.3.7.4 Deafness and Dystonia Syndrome (Mohr-Tranebjaerg Syndrome) -- 7.3.8 Conclusion -- 7.4 Mitochondrial DNA and Aging -- 7.4.1 Introduction -- 7.4.2 Accumulation of mtDNA Damage and Normal Aging -- 7.4.3 Neurodegenerative Diseases -- 7.4.3.1 Parkinson's Disease -- 7.4.3.2 Alzheimer's Disease -- 7.4.3.3 Huntington's Disease -- 7.4.3.4 Amyotrophic Lateral Sclerosis (ALS) -- 7.5 Mitochondria and Apoptosis -- 7.6 Fungal Senescence -- 7.7 Cytoplasmic Male Sterility in Plants -- References -- 8 Mitochondrial DNA Sequencing and Anthropology -- 8.1 Introduction -- 8.2 Human Evolution -- 8.3 Primate Evolution -- 8.4 Human Y Chromosome Variation -- 8.5 Forensic Applications -- 8.6 Future Challenges -- References -- 9 Mitochondria and Pharmacology -- 9.1 Introduction -- References -- Index.