Mitochondria

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1. Verfasser: Scheffler, Immo E. (VerfasserIn)
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Veröffentlicht: Hoboken, NJ Wiley-Liss 2008
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adam_text CONTENTS Preface xi Preface to First Edition xv 1 History 1 References, 6 2 Evolutionary Origin of Mitochondria 7 References, 15 3 Structure and Morphology. Integration into the Cell 18 3.1 Structure and Morphology, 19 3.2 Integration into the Cell, 30 3.2.1 Distribution in the Су toso!, ЗО 3.2.2 Interaction with Cytoskeleton, 34 3.3 The Dynamics of Mitochondnal Morphology, 37 3.3.1 Mitochondnal Shape Changes, 37 3.3.1.1 Fission. 40 3.3.1.2 Fusion, 42 3.3.2 Distribution During Cell Division, 46 3.3.3 During Cell Differentiation, 51 3.3.4 Turnover and Degradation, 54 3.3.5 Mitochondrial Alterations in Apoptosis, 55 3.3.6 Unsolved Problems for the Future, 55 References, 56 vj CONTENTS 4 Biogenesis of Mitochondria 60 4.1 The Mitochondrial Genome, 61 4.1.1 Introduction, 61 4.1.2 The Mitochondrial Genome in Metazoans, 63 4.1.3 The Mitochondrial Genome in Plants, 68 4.1.4 The Mitochondrial Genome in Fungi, 72 4.1.5 The Mitochondrial Genome in Kinetoplastid Protozoa, 75 4.1.6 Mitochondrial Plasmids, 79 4.1.6.1 Fungal Senescence, 80 4.1.6.2 Phytopathogenicity, 81 4.1.6.3 Cytoplasmic Male Sterility (CMS), 81 4.2 Nuclear Genes Encoding Mitochondrial Proteins, 81 4.2.1 Enzymes Required for Maintenance and Expression of the Mitochondrial Genome, 82 4.2.2 Nucleo-mitochondrial Interactions, 85 4.2.2.1 Introduction, 85 4.2.2.2 In Yeast, Saccharomyces cerevisiae, 86 4.2.2.3 Regulation of Nuclear Respiratory Genes in Mammalian Cells, 91 4.2.2.4 Co-evolution of Nuclear and Mitochondrial Genomes, 95 4.3 Replication and Maintenance of Mitochondrial DNA, 96 4.3.1 DNA Replication in Mammalian Mitochondria, 96 4.3.2 mtDNA Repair in Mammalian Mitochondria, 104 4.3.3 Recombination in Mammalian Mitochondria, 105 4.3.4 mtDNA Maintenance and Replication in Other Organisms, 105 4.4 Transcription of Mitochondrial DNA-RNA Metabolism, 105 4.4.1 Transcription in Mammalian Mitochondria, 105 4.4.2 Transcription of mtDNA in the Yeast Saccharomyces cerevisiae, 111 4.4.3 Transcription of mtDNA in Plant Mitochondria, 111 4.4.4 Transcriptional Termination, 113 4.4.5 RNA Processing in Mitochondria, 113 4.4.6 RNA Editing in Kinetoplastid Protozoa, 116 4.4.7 Editing in Plant Mitochondria, 121 4.4.8 Control of mRNA Levels by Turnover, 123 4.5 Translation of Mitochondrial mRNAs, 126 4.5.1 Introduction, 126 4.5.2 Codon Usage and tRNA Structure, 126 4.5.3 Mitochondrial Ribosomes, 128 4.5.4 Cis-Acting Elements. 132 4.5.5 Translation Factors, 133 4.6 Protein Import into Mitochondria. 141 CONTENTS vii 4.6.1 Mitochondrial Targeting of Proteins, 141 4.6.2 The Protein Import Machinery of Mitochondria, 145 4.7 Import of Transfer RNA into Mitochondria, 152 4.8 Regulated Protein Degradation in Mitochondria, 153 References, 156 5 Mitochondrial Electron Transfer and Oxidative Phosphorylation 168 5.1 Historical Introduction, 169 5.2 The Electron Transport Chain, 173 5.2.1 The Biochemical Components, 173 5.2.2 Physical Separation of the Complexes of the ETC, 179 5.2.2.1 Biochemical Fractionations, 179 5.2.2.2 Supercomplexes, 182 5.2.3 Introduction to Bioenergetics, 184 5.2.4 Complex I, 185 5.2.5 Complex II, 195 5.2.5.1 Nuclear Versus Mitochondrial Location of Complex II Genes, 200 5.2.6 Complex III, 202 5.2.7 Complex IV, 210 5.2.8 The Assembly of the Electron Transport Chain Complexes, 220 5.3 Electron Transport in Other Organisms, 224 5.3.1 NAD(P)H Dehydrogenases. 224 5.3.2 A Cyanide-Insensitive Electron Pathway, 225 5.3.3 NADH Oxidation in Yeasts, 226 5.3.4 Energy Metabolism and NADH Oxidation in Trypanosomes, 227 5.4 The Chemiosmotic Hypothesis, 228 5.4.1 The Mitchell Hypothesis, 228 5.4.2 The Q Cycle, 237 5.4.3 Probing the Mitochondrial Membrane Potential with Fluorescent Dyes. 239 5.5 ATP Synthase (F,Fn -ATPase), 244 5.5.1 Introduction, 244 5.5.2 Х -Ray Structure, 247 5.5.3 ATP Synthesis and Catalytic Mechanisms. 249 5.5.4 The Fo Subcomplex and Proton Flow, 253 5.5.5 Assembly of Complex V and Dimerization, 257 5.6 Control of Respiration and Oxidative Phosphorylation, 259 5.6.1 General Considerations, 259 5.6.1.1 The Role of Substrates. 261 5.6.2 The Uncoupling Proteins in Warm-Blooded Animals, 266 5.6.3 Uncoupling in Other Organisms, 271 УІИ CONTENTS 5.6.3.1 In Saccharomyces cerevisiae, 271 5.6.3.2 In Plants, 271 5.7 Reactive Oxygen Species, 272 5.8 Nitric Oxide (NO), 277 5.9 The Role of Specific Lipids, 279 References, 281 6 Metabolic Pathways Inside Mitochondria 298 6.1 Introduction, 299 6.2 The Krebs Cycle, 299 6.3 Fatty Acid Metabolism, 304 6.4 The Urea Cycle, 309 6.5 Biosynthesis of Heme, 309 6.6 Cardiolipin and Lipid Biosynthesis/Metabolism, 314 6.7 Biosynthesis of Ubiquinol (Coenzyme Q), 319 6.8 Biosynthesis of Fe-S Centers, 324 6.9 Transport of Small Solutes into and out of Mitochondria, 329 6.9.1 Introduction, 329 6.9.2 Porin Alias VDAC, 330 6.9.3 The ADP/ATP Translocator, 331 6.9.4 The Mitochondrial Carrier Protein Family, 334 6.9.5 Cation Transport, 335 6.9.5.1 Transport of Calcium and Its Physiological Role, 337 6.9.6 The Mitochondrial Permeability Transition, 339 References, 341 7 Mitochondrial Mutations and Disease 345 7.1 General Introduction, 346 7.2 In Cell Culture, 348 7.2.1 Mitochondrial Mutations in Microorganisms, 348 7.2.2 Mitochondrial Mutations in Mammalian Cells in Culture, 350 7.3 Molecular Genetics of Human Mitochondrial Diseases, 354 7.3.1 Introduction, 354 7.3.2 Maternal Versus Sporadic Inheritance, 355 7.3.3 Mapping mtDNA Deletions/Rearrangements, 358 7.3.4 mtDNA Point Mutations and Maternal Inheritance, 359 7.3.5 Mitochondria and Oogénesis, 362 7.3.6 Clinical Aspects of Mitochondrial DNA Mutations, 366 7.3.6.1 MtDNA Deletions: Kearns-Sayre Syndrome and Pearson Syndrome, 367 7.3.6.2 Familial Mitochondrial DNA Depletion, 369 7.3.6.3 Point Mutations, 369 7.3.7 Nuclear Mutations and Mitochondrial Disease, 373 7.3.7.1 Defective Electron Transport Chain, 373 CONTENTS ix 7.3.7.2 MtDNA Maintenance and Replication, 375 7.3.7.3 Friedreich s Ataxia, 376 7.3.7.4 Deafness and Dystonia Syndrome (Mohr-Tranebjaerg Syndrome), 376 7.3.8 Conclusion, 377 7.4 Mitochondrial DNA and Aging, 377 7.4.1 Introduction, 377 7.4.2 Accumulation of mtDNA Damage and Normal Aging, 380 7.4.3 Neurodegenerative Diseases, 383 7.4.3.1 Parkinson s Disease, 384 7.4.3.2 Alzheimer s Disease, 386 7.4.3.3 Huntington s Disease, 387 7.4.3.4 Amyotrophic Lateral Sclerosis (ALS), 389 7.5 Mitochondria and Apoptosis, 389 7.6 Fungal Senescence, 398 7.7 Cytoplasmic Male Sterility in Plants, 400 References, 404 8 Mitochondrial DNA Sequencing and Anthropology 417 8.1 Introduction, 418 8.2 Human Evolution, 419 8.3 Primate Evolution, 426 8.4 Human Y Chromosome Variation, 428 8.5 Forensic Applications, 430 8.6 Future Challenges, 434 References, 434 9 Mitochondria and Pharmacology 437 9.1 Introduction, 437 References, 438 Index 441
adam_txt CONTENTS Preface xi Preface to First Edition xv 1 History 1 References, 6 2 Evolutionary Origin of Mitochondria 7 References, 15 3 Structure and Morphology. Integration into the Cell 18 3.1 Structure and Morphology, 19 3.2 Integration into the Cell, 30 3.2.1 Distribution in the Су toso!, ЗО 3.2.2 Interaction with Cytoskeleton, 34 3.3 The Dynamics of Mitochondnal Morphology, 37 3.3.1 Mitochondnal Shape Changes, 37 3.3.1.1 Fission. 40 3.3.1.2 Fusion, 42 3.3.2 Distribution During Cell Division, 46 3.3.3 During Cell Differentiation, 51 3.3.4 Turnover and Degradation, 54 3.3.5 Mitochondrial Alterations in Apoptosis, 55 3.3.6 Unsolved Problems for the Future, 55 References, 56 vj CONTENTS 4 Biogenesis of Mitochondria 60 4.1 The Mitochondrial Genome, 61 4.1.1 Introduction, 61 4.1.2 The Mitochondrial Genome in Metazoans, 63 4.1.3 The Mitochondrial Genome in Plants, 68 4.1.4 The Mitochondrial Genome in Fungi, 72 4.1.5 The Mitochondrial Genome in Kinetoplastid Protozoa, 75 4.1.6 Mitochondrial Plasmids, 79 4.1.6.1 Fungal Senescence, 80 4.1.6.2 Phytopathogenicity, 81 4.1.6.3 Cytoplasmic Male Sterility (CMS), 81 4.2 Nuclear Genes Encoding Mitochondrial Proteins, 81 4.2.1 Enzymes Required for Maintenance and Expression of the Mitochondrial Genome, 82 4.2.2 Nucleo-mitochondrial Interactions, 85 4.2.2.1 Introduction, 85 4.2.2.2 In Yeast, Saccharomyces cerevisiae, 86 4.2.2.3 Regulation of Nuclear Respiratory Genes in Mammalian Cells, 91 4.2.2.4 Co-evolution of Nuclear and Mitochondrial Genomes, 95 4.3 Replication and Maintenance of Mitochondrial DNA, 96 4.3.1 DNA Replication in Mammalian Mitochondria, 96 4.3.2 mtDNA Repair in Mammalian Mitochondria, 104 4.3.3 Recombination in Mammalian Mitochondria, 105 4.3.4 mtDNA Maintenance and Replication in Other Organisms, 105 4.4 Transcription of Mitochondrial DNA-RNA Metabolism, 105 4.4.1 Transcription in Mammalian Mitochondria, 105 4.4.2 Transcription of mtDNA in the Yeast Saccharomyces cerevisiae, 111 4.4.3 Transcription of mtDNA in Plant Mitochondria, 111 4.4.4 Transcriptional Termination, 113 4.4.5 RNA Processing in Mitochondria, 113 4.4.6 RNA Editing in Kinetoplastid Protozoa, 116 4.4.7 Editing in Plant Mitochondria, 121 4.4.8 Control of mRNA Levels by Turnover, 123 4.5 Translation of Mitochondrial mRNAs, 126 4.5.1 Introduction, 126 4.5.2 Codon Usage and tRNA Structure, 126 4.5.3 Mitochondrial Ribosomes, 128 4.5.4 Cis-Acting Elements. 132 4.5.5 Translation Factors, 133 4.6 Protein Import into Mitochondria. 141 CONTENTS vii 4.6.1 Mitochondrial Targeting of Proteins, 141 4.6.2 The Protein Import Machinery of Mitochondria, 145 4.7 Import of Transfer RNA into Mitochondria, 152 4.8 Regulated Protein Degradation in Mitochondria, 153 References, 156 5 Mitochondrial Electron Transfer and Oxidative Phosphorylation 168 5.1 Historical Introduction, 169 5.2 The Electron Transport Chain, 173 5.2.1 The Biochemical Components, 173 5.2.2 Physical Separation of the Complexes of the ETC, 179 5.2.2.1 Biochemical Fractionations, 179 5.2.2.2 Supercomplexes, 182 5.2.3 Introduction to Bioenergetics, 184 5.2.4 Complex I, 185 5.2.5 Complex II, 195 5.2.5.1 Nuclear Versus Mitochondrial Location of Complex II Genes, 200 5.2.6 Complex III, 202 5.2.7 Complex IV, 210 5.2.8 The Assembly of the Electron Transport Chain Complexes, 220 5.3 Electron Transport in Other Organisms, 224 5.3.1 NAD(P)H Dehydrogenases. 224 5.3.2 A Cyanide-Insensitive Electron Pathway, 225 5.3.3 NADH Oxidation in Yeasts, 226 5.3.4 Energy Metabolism and NADH Oxidation in Trypanosomes, 227 5.4 The Chemiosmotic Hypothesis, 228 5.4.1 The Mitchell Hypothesis, 228 5.4.2 The Q Cycle, 237 5.4.3 Probing the Mitochondrial Membrane Potential with Fluorescent Dyes. 239 5.5 ATP Synthase (F,Fn'-ATPase), 244 5.5.1 Introduction, 244 5.5.2 Х -Ray Structure, 247 5.5.3 ATP Synthesis and Catalytic Mechanisms. 249 5.5.4 The Fo Subcomplex and Proton Flow, 253 5.5.5 Assembly of Complex V and Dimerization, 257 5.6 Control of Respiration and Oxidative Phosphorylation, 259 5.6.1 General Considerations, 259 5.6.1.1 The Role of Substrates. 261 5.6.2 The Uncoupling Proteins in Warm-Blooded Animals, 266 5.6.3 Uncoupling in Other Organisms, 271 УІИ CONTENTS 5.6.3.1 In Saccharomyces cerevisiae, 271 5.6.3.2 In Plants, 271 5.7 Reactive Oxygen Species, 272 5.8 Nitric Oxide (NO), 277 5.9 The Role of Specific Lipids, 279 References, 281 6 Metabolic Pathways Inside Mitochondria 298 6.1 Introduction, 299 6.2 The Krebs Cycle, 299 6.3 Fatty Acid Metabolism, 304 6.4 The Urea Cycle, 309 6.5 Biosynthesis of Heme, 309 6.6 Cardiolipin and Lipid Biosynthesis/Metabolism, 314 6.7 Biosynthesis of Ubiquinol (Coenzyme Q), 319 6.8 Biosynthesis of Fe-S Centers, 324 6.9 Transport of Small Solutes into and out of Mitochondria, 329 6.9.1 Introduction, 329 6.9.2 Porin Alias VDAC, 330 6.9.3 The ADP/ATP Translocator, 331 6.9.4 The Mitochondrial Carrier Protein Family, 334 6.9.5 Cation Transport, 335 6.9.5.1 Transport of Calcium and Its Physiological Role, 337 6.9.6 The Mitochondrial Permeability Transition, 339 References, 341 7 Mitochondrial Mutations and Disease 345 7.1 General Introduction, 346 7.2 In Cell Culture, 348 7.2.1 Mitochondrial Mutations in Microorganisms, 348 7.2.2 Mitochondrial Mutations in Mammalian Cells in Culture, 350 7.3 Molecular Genetics of Human Mitochondrial Diseases, 354 7.3.1 Introduction, 354 7.3.2 Maternal Versus Sporadic Inheritance, 355 7.3.3 Mapping mtDNA Deletions/Rearrangements, 358 7.3.4 mtDNA Point Mutations and Maternal Inheritance, 359 7.3.5 Mitochondria and Oogénesis, 362 7.3.6 Clinical Aspects of Mitochondrial DNA Mutations, 366 7.3.6.1 MtDNA Deletions: Kearns-Sayre Syndrome and Pearson Syndrome, 367 7.3.6.2 Familial Mitochondrial DNA Depletion, 369 7.3.6.3 Point Mutations, 369 7.3.7 Nuclear Mutations and Mitochondrial Disease, 373 7.3.7.1 Defective Electron Transport Chain, 373 CONTENTS ix 7.3.7.2 MtDNA Maintenance and Replication, 375 7.3.7.3 Friedreich's Ataxia, 376 7.3.7.4 Deafness and Dystonia Syndrome (Mohr-Tranebjaerg Syndrome), 376 7.3.8 Conclusion, 377 7.4 Mitochondrial DNA and Aging, 377 7.4.1 Introduction, 377 7.4.2 Accumulation of mtDNA Damage and Normal Aging, 380 7.4.3 Neurodegenerative Diseases, 383 7.4.3.1 Parkinson's Disease, 384 7.4.3.2 Alzheimer's Disease, 386 7.4.3.3 Huntington's Disease, 387 7.4.3.4 Amyotrophic Lateral Sclerosis (ALS), 389 7.5 Mitochondria and Apoptosis, 389 7.6 Fungal Senescence, 398 7.7 Cytoplasmic Male Sterility in Plants, 400 References, 404 8 Mitochondrial DNA Sequencing and Anthropology 417 8.1 Introduction, 418 8.2 Human Evolution, 419 8.3 Primate Evolution, 426 8.4 Human Y Chromosome Variation, 428 8.5 Forensic Applications, 430 8.6 Future Challenges, 434 References, 434 9 Mitochondria and Pharmacology 437 9.1 Introduction, 437 References, 438 Index 441
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spelling Scheffler, Immo E. Verfasser aut
Mitochondria Immo E. Scheffler
2. ed.
Hoboken, NJ Wiley-Liss 2008
XVIII, 462 S., [6] Bl. Ill., graph. Darst.
txt rdacontent
n rdamedia
nc rdacarrier
Maladies mitochondriales
Mitochondries
DNA, Mitochondrial
Genes, Mitochondrial
Mitochondria
Mitochondrial pathology
Mitochondrium (DE-588)4124939-2 gnd rswk-swf
Mitochondrium (DE-588)4124939-2 s
DE-604
Digitalisierung UB Regensburg application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=016081645&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis
spellingShingle Scheffler, Immo E.
Mitochondria
Maladies mitochondriales
Mitochondries
DNA, Mitochondrial
Genes, Mitochondrial
Mitochondria
Mitochondrial pathology
Mitochondrium (DE-588)4124939-2 gnd
subject_GND (DE-588)4124939-2
title Mitochondria
title_auth Mitochondria
title_exact_search Mitochondria
title_exact_search_txtP Mitochondria
title_full Mitochondria Immo E. Scheffler
title_fullStr Mitochondria Immo E. Scheffler
title_full_unstemmed Mitochondria Immo E. Scheffler
title_short Mitochondria
title_sort mitochondria
topic Maladies mitochondriales
Mitochondries
DNA, Mitochondrial
Genes, Mitochondrial
Mitochondria
Mitochondrial pathology
Mitochondrium (DE-588)4124939-2 gnd
topic_facet Maladies mitochondriales
Mitochondries
DNA, Mitochondrial
Genes, Mitochondrial
Mitochondria
Mitochondrial pathology
Mitochondrium
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