Mitochondria

by Scheffler, Immo E.

• ISBN13: 9780470040737
• ISBN10: 0470040734
• Edition: 2nd
• Format: Hardcover
• Copyright: 2007-11-27
• Publisher: Wiley-Liss

Summary

"This volume inspires. It certainly will be much appreciated by cell biologists all over the world." Quarterly Review of Biology, March 2009This book is the eagerly awaited second edition of the best-selling Mitochondria, a book widely acknowledged as the first modern, truly comprehensive authored work on the important, scientifically fundamental topic of the cellular organelles known as mitochondria.This new edition brings readers completely up to date on the many significant findings that have occurred in the eight years since the book was first published. As in that seminal first edition, the second edition tackles the biochemistry, genetics, and pathology of mitochondria in different organisms. The new edition provides thorough updates of all literature concerning this vital organelle, its functions, ongoing research surrounding it, and its importance vis-a-vis a broad range of issues in cellular and molecular biology. The book includes detailed descriptions of current and developing technologies around mitochondrial research and discovery, and highlights subjects that are growing, such as the use of proteomics.This book is an invaluable resource for all geneticists, biologists, and educators in life sciences. It is also of interest for advanced students in genetics and molecular biology.

Author Biography

Immo E. Scheffler, PhD, is Professor in the Division of Biology (Molecular Biology Section) at the University of California, San Diego. In addition to this well-received book, Dr. Scheffler has more than 100 original publications in scientific literature and has written numerous book chapters.

Table of Contents

Preface	p. xi
Preface to First Edition	p. xv
History	p. 1
References	p. 6
Evolutionary Origin of Mitochondria	p. 7
References	p. 15
Structure and Morphology. Integration into the Cell	p. 18
Structure and Morphology	p. 19
Integration into the Cell	p. 30
Distribution in the Cytosol	p. 30
Interaction with Cytoskeleton	p. 34
The Dynamics of Mitochondrial Morphology	p. 37
Mitochondrial Shape Changes	p. 37
Fission	p. 40
Fusion	p. 42
Distribution During Cell Division	p. 46
During Cell Differentiation	p. 51
Turnover and Degradation	p. 54
Mitochondrial Alterations in Apoptosis	p. 55
Unsolved Problems for the Future	p. 55
References	p. 56
Biogenesis of Mitochondria	p. 60
The Mitochondrial Genome	p. 61
Introduction	p. 61
The Mitochondrial Genome in Metazoans	p. 63
The Mitochondrial Genome in Plants	p. 68
The Mitochondrial Genome in Fungi	p. 72
The Mitochondrial Genome in Kinetoplastid Protozoa	p. 75
Mitochondrial Plasmids	p. 79
Fungal Senescence	p. 80
Phytopathogenicity	p. 81
Cytoplasmic Male Sterility (CMS)	p. 81
Nuclear Genes Encoding Mitochondrial Proteins	p. 81
Enzymes Required for Maintenance and Expression of the Mitochondrial Genome	p. 82
Nucleo-mitochondrial Interactions	p. 85
Introduction	p. 85
In Yeast, Saccharomyces cerevisiae	p. 86
Regulation of Nuclear Respiratory Genes in Mammalian Cells	p. 91
Co-evolution of Nuclear and Mitochondrial Genomes	p. 95
Replication and Maintenance of Mitochondrial DNA	p. 96
DNA Replication in Mammalian Mitochondria	p. 96
mtDNA Repair in Mammalian Mitochondria	p. 104
Recombination in Mammalian Mitochondria	p. 105
mtDNA Maintenance and Replication in Other Organisms	p. 105
Transcription of Mitochondrial DNA-RNA Metabolism	p. 105
Transcription in Mammalian Mitochondria	p. 105
Transcription of mtDNA in the Yeast Saccharomyces cerevisiae	p. 111
Transcription of mtDNA in Plant Mitochondria	p. 111
Transcriptional Termination	p. 113
RNA Processing in Mitochondria	p. 113
RNA Editing in Kinetoplastid Protozoa	p. 116
Editing in Plant Mitochondria	p. 121
Control of mRNA Levels by Turnover	p. 123
Translation of Mitochondrial mRNAs	p. 126
Introduction	p. 126
Codon Usage and tRNA Structure	p. 126
Mitochondrial Ribosomes	p. 128
Cis-Acting Elements	p. 132
Translation Factors	p. 133
Protein Import into Mitochondria	p. 141
Mitochondrial Targeting of Proteins	p. 141
The Protein Import Machinery of Mitochondria	p. 145
Import of Transfer RNA into Mitochondria	p. 152
Regulated Protein Degradation in Mitochondria	p. 153
References	p. 156
Mitochondrial Electron Transfer and Oxidative Phosphorylation	p. 168
Historical Introduction	p. 169
The Electron Transport Chain	p. 173
The Biochemical Components	p. 173
Physical Separation of the Complexes of the ETC	p. 179
Biochemical Fractionations	p. 179
Supercomplexes	p. 182
Introduction to Bioenergetics	p. 184
Complex I	p. 185
Complex II	p. 195
Nuclear Versus Mitochondrial Location of Complex II Genes	p. 200
Complex III	p. 202
Complex IV	p. 210
The Assembly of the Electron Transport Chain Complexes	p. 220
Electron Transport in Other Organisms	p. 224
NAD(P)H Dehydrogenases	p. 224
A Cyanide-Insensitive Electron Pathway	p. 225
NADH Oxidation in Yeasts	p. 226
Energy Metabolism and NADH Oxidation in Trypanosomes	p. 227
The Chemiosmotic Hypothesis	p. 228
The Mitchell Hypothesis	p. 228
The Q Cycle	p. 237
Probing the Mitochondrial Membrane Potential with Fluorescent Dyes	p. 239
ATP Synthase (F[subscript 1]F[subscript 0]-ATPase)	p. 244
Introduction	p. 244
X-Ray Structure	p. 247
ATP Synthesis and Catalytic Mechanisms	p. 249
The F[subscript 0] Subcomplex and Proton Flow	p. 253
Assembly of Complex V and Dimerization	p. 257
Control of Respiration and Oxidative Phosphorylation	p. 259
General Considerations	p. 259
The Role of Substrates	p. 261
The Uncoupling Proteins in Warm-Blooded Animals	p. 266
Uncoupling in Other Organisms	p. 271
In Saccharomyces cerevisiae	p. 271
In Plants	p. 271
Reactive Oxygen Species	p. 272
Nitric Oxide (NO)	p. 277
The Role of Specific Lipids	p. 279
References	p. 281
Metabolic Pathways Inside Mitochondria	p. 298
Introduction	p. 299
The Krebs Cycle	p. 299
Fatty Acid Metabolism	p. 304
The Urea Cycle	p. 309
Biosynthesis of Heme	p. 309
Cardiolipin and Lipid Biosynthesis/Metabolism	p. 314
Biosynthesis of Ubiquinol (Coenzyme Q)	p. 319
Biosynthesis of Fe-S Centers	p. 324
Transport of Small Solutes into and out of Mitochondria	p. 329
Introduction	p. 329
Porin Alias VDAC	p. 330
The ADP/ATP Translocator	p. 331
The Mitochondrial Carrier Protein Family	p. 334
Cation Transport	p. 335
Transport of Calcium and Its Physiological Role	p. 337
The Mitochondrial Permeability Transition	p. 339
References	p. 341
Mitochondrial Mutations and Disease	p. 345
General Introduction	p. 346
In Cell Culture	p. 348
Mitochondrial Mutations in Microorganisms	p. 348
Mitochondrial Mutations in Mammalian Cells in Culture	p. 350
Molecular Genetics of Human Mitochondrial Diseases	p. 354
Introduction	p. 354
Maternal Versus Sporadic Inheritance	p. 355
Mapping mtDNA Deletions/Rearrangements	p. 358
mtDNA Point Mutations and Maternal Inheritance	p. 359
Mitochondria and Oogenesis	p. 362
Clinical Aspects of Mitochondrial DNA Mutations	p. 366
MtDNA Deletions: Kearns-Sayre Syndrome and Pearson Syndrome	p. 367
Familial Mitochondrial DNA Depletion	p. 369
Point Mutations	p. 369
Nuclear Mutations and Mitochondrial Disease	p. 373
Defective Electron Transport Chain	p. 373
MtDNA Maintenance and Replication	p. 375
Friedreich's Ataxia	p. 376
Deafness and Dystonia Syndrome (Mohr-Tranebjaerg Syndrome)	p. 376
Conclusion	p. 377
Mitochondrial DNA and Aging	p. 377
Introduction	p. 377
Accumulation of mtDNA Damage and Normal Aging	p. 380
Neurodegenerative Diseases	p. 383
Parkinson's Disease	p. 384
Alzheimer's Disease	p. 386
Huntington's Disease	p. 387
Amyotrophic Lateral Sclerosis (ALS)	p. 389
Mitochondria and Apoptosis	p. 389
Fungal Senescence	p. 398
Cytoplasmic Male Sterility in Plants	p. 400
References	p. 404
Mitochondrial DNA Sequencing and Anthropology	p. 417
Introduction	p. 418
Human Evolution	p. 419
Primate Evolution	p. 426
Human Y Chromosome Variation	p. 428
Forensic Applications	p. 430
Future Challenges	p. 434
References	p. 434
Mitochondria and Pharmacology	p. 437
Introduction	p. 437
References	p. 438
Index	p. 441
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