Mitochondrial function and biogenesis: with 8 tables
Saved in:
| Format: | Book |
|---|---|
| Language: | English |
| Published: |
Berlin [u.a.]
Springer
2004
|
| Series: | Topics in current genetics
8 |
| Subjects: | |
| Online Access: | Inhaltsverzeichnis |
| Physical Description: | XVI, 333 S. Ill., graph. Darst. |
| ISBN: | 3540214895 |
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Record in the Search Index
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| adam_text | Table of contents
Mitochondrial genome evolution: the origin of mitochondria and of
eukaryotes 1
Rachel B. Bevan and B. Franz Lang 1
Abstract 1
1 Introduction 1
2 Evolution of mitochondrial genomes and genes: anything is possible 2
2.1 The perplexing diversity of mitochondrial genome architecture 3
2.2 Unusual mitochondrial gene structure and gene expression 5
2.3 Past and current gene loss and gene emigration from the
mitochondrial to the nuclear genome 7
2.4 Immigration of genes to mitochondria, and horizontal gene
transfer among plant mitochondrial genomes 11
2.5 Possible mechanisms of gene migration 13
3 Mitochondrial genome diversity in major eukaryotic groups 14
3.1 Animals 14
3.2 Fungi 15
3.3 Plants and green algae 16
3.4Protists 17
4 Value of mitochondrial sequences for phylogenetic inference 18
5 Origin and evolution of mitochondria and of the eukaryotes 19
5.1 Eubacterial ancestry of mitochondria 19
5.2 Serial endosymbiosis, or metabolic syntrophy model of
mitochondrial origin? 21
6 Concluding remarks 23
Acknowledgements 25
References 25
Glossary 34
Mitochondrial biogenesis: Protein import into and across the outer
membrane 37
Doron Rapaport and Frank E. Nargang 37
Abstract 37
1 Targeting of mitochondrial precursor proteins from the cytosol to the
mitochondria 37
2 The translocase of the outer membrane as the gate to the organelle 38
2.1 Structure and composition of the TOM complex 38
2.2 Tom40 is the most important component of the TOM complex 39
3 Translocation of precursors with a presequence across the TOM
complex 40
3.1 Interactions of presequences with various Tom components 40
3.2 The driving force for translocation of presequences across the
outer membrane 42
4 Interactions of the TOM complex with precursors containing internal
targeting signals 42
4.1 Import of inner membrane and intermembrane space proteins 43
5 Import of outer membrane proteins 46
5.1 N terminal anchored proteins 47
5.2 Tail anchored proteins 48
5.3 P barrel proteins 50
Acknowledgments 51
References 51
Mitochondrial biogenesis. Protein import into and across the inner
membrane 59
Sean P. Curran and Carla M. Koehler 59
Abstract 59
1 Introduction 59
2 Targeting to the mitochondria 62
2.1 Characteristics of targeting sequences 62
2.2 Proteins localized to more than one compartment 63
3 TheTIM23 translocon 63
3.1 Translocon composition 63
3.2 Energetics of the TIM23 translocon 65
4 The TIM22 translocon 67
4.1 Evolution of the TIM complexes 67
4.2 TIM22 translocon composition 67
4.3 Soluble tiny Tim proteins in the intermembrane space 68
4.4 Five stages of translocation 69
5 Mitochondrial protein export pathways 71
6 Perspectives 72
Acknowledgements 72
References 73
Mitochondrial tRNA editing 81
Jens Schuster and Mario Mori 81
Abstract 81
1 Introduction 81
2 5 Nucleotide replacement: restoring the acceptor stem 83
3 3 Nucleotide addition: back to completion 85
4Nucleotide insertion: U and C 88
5 Base conversion: processing prerequisite or identity switch 89
6 Perspective 92
Acknowledgements 93
References 93
Protein quality control in mitochondria and neurodegeneration in
hereditary spastic paraplegia 97
Peter Bross, Elena I. Rugarli, Giorgio Casari, and Thomas Langer 97
Abstract 97
1 Introduction 97
2 Protein folding in mitochondria 99
3 Quality control of mitochondrial proteins 101
3.1 Quality control of matrix proteins 102
3.2 Quality control of inner membrane proteins 103
4 Pathogenesis of hereditary spastic paraplegia (HSP) 106
4.1 The pathogenic mechanism of HSP due to lack of paraplegin 108
4.2 Disease associated mutation in HSP60 109
4.3 The role of impaired protein quality control in mitochondria in
pathogenesis of HSP 111
5 Mitochondrial malfunction and axonal transport disturbances in
HSP: Is there a common denominator? 113
References 114
Defects in assembly of cytochrome oxidase: roles in
mitochondrial disease 123
Fiona A. Punter and D. Moira Glerum 123
Abstract 123
1 Introduction 123
2 Structure and function of cytochrome oxidase 124
3 Assembly of cytochrome oxidase 124
3.1 PET genes Involved in holoenzyme assembly 127
3.2 Heme A biosynthesis in COX assembly 128
3.3 Copper provision for COX assembly 129
3.4 COX assembly in human cells 133
4 Human cytochrome oxidase deficiency 135
4.1 Mutations in mtDNA causing COX deficiency 136
4.2 Mutations in COX assembly factor genes causing COX
deficiency 137
5 Discussion and conclusions 140
Acknowledgements 141
References 141
Function and dysfunction of the oxidative phosphorylation system 149
Leo G. J. Nijtmans, Cristina Ugalde, Lambert P. van den Heuvel,
and Jan A. M. Smeitink 149
Abstract 149
1 Introduction 149
2 The OXPHOS system 151
3 Complex 1 152
3.1 Complex I function 152
3.2 Complex I structure 152
3.3 Subunit composition 153
3.4 Prosthetic groups 153
3.5 The core subunits 154
3.6 The accessory subunits 155
3.7 The biogenesis of mitochondrial complex 1 156
3.8 Complex I disorders 157
4 Complex II 158
5 Complex III 159
6 Complex IV 160
7 Complex V 162
8 Respiratory chain organisation 163
9 The price of mitochondrial DNA 164
10 Reactive oxygen species production of the OXHPOS system 165
11 Concluding remarks 167
Acknowledgements 167
References 167
MtDNA maintenance and stability genes: MNGIE and mtDNA depletion
syndromes 177
Michio Hirano, Ramon Marti, Maya R. Vila, Yutaka Nishigaki 177
Abstract 177
1 Introduction 177
2 Mitochondrial DNA structure 179
3 Replication of MtDNA 179
4 MtDNA repair 181
5 MtDNA recombination 183
6 Mitochondrial nucleoside/nucleotide metabolism 183
7 Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) 185
8 MtDNA depletion 189
9 Conclusion 191
Acknowledgements 192
References 192
Abbreviations 199
Protein translocation into mammalian mitochondria and its role in the
development of human mitochondrial disorders 201
Sabine Hofmann and Matthias F. Bauer 201
Abstract 201
1 Components and mechanisms of protein import into mammalian
mitochondria 201
2 The mammalian TOM machinery 204
3 The mammalian translocases of the mitochondrial inner membrane 207
3.1 The TIM23 machinery 207
3.2 The TIM22 machinery 209
4 The family of the small Tim proteins of intermembrane space 211
5 Mohr Tranebjaerg syndrome is caused by a defect in a mitochondrial
protein import pathway 213
5.1 Clinical presentation of the Mohr Tranebjaerg syndrome 213
5.2 Genetics of Mohr Tranebjaerg syndrome 214
5.3 Mitochondrial dysfunction in Mohr Tranebjaerg syndrome 216
6 Concluding remarks 218
Acknowledgements 218
References 218
Mitochondrial fission and fusion machineries 227
Lorena Griparic, Brian Head, and Alexander M. van der Bliek 227
Abstract 227
1 Introduction 227
2 Visualization of mitochondrial fission and fusion 228
3 Mitochondrial division 229
3.1 Role of a dynamin related protein in mitochondrial division 230
3.2 Other proteins that contribute to mitochondrial outer membrane
division 231
3.3 Mitochondrial inner membrane division 234
3.4 Mitochondrial division during apoptosis 235
4 Fusion between mitochondria 236
4.1 Drosophila fuzzy onions, its yeast homologue Fzolp and
mammalian Mitofusins 236
4.2 Mgml, a dynamin family member implicated in
mitochondrial fusion 238
4.3 Ugol, a yeast accessory to Fzol and Mgml 241
4.4 Mdm30, a possible link between mitochondrial fusion and
ubiquitination 242
4.5 A v SNARE and a SNAP binding protein that might affect
mitochondrial fusion 242
5 Future directions 242
Acknowledgements 243
References 243
VDAC function in a cellular context 251
Michael Forte 251
Abstract 251
1 Introduction 251
2 VDAC channel the basics 252
3 Genetics of mammalian VDACS 255
4 Mitochondria, VDAC, and Ca2+ homeostasis 256
5 VDAC and cell death 258
6 VDAC and the permeability transition pore 260
7 Conclusions 261
References 262
Lipid synthesis and transport in mitochondrial biogenesis 267
Dennis R. Voelker 267
Abstract 267
1 Introduction 267
2 Lipid composition and function 269
2.1 Phospholipid structure 269
2.2 Phosphatidylcholine 269
2.3 Phosphatidylethanolamine 271
2.4 Phosphatidylserine 271
2.5 Phosphatidylglycerol 271
2.6 Cardiolipin 272
2.7 Other phospholipids 273
2.8Sterols 275
3 Cellular lipid synthesis and the mitochondria 275
3.1 Non mitochondrial phospholipid synthesis 275
3.2 Mitochondrial phospholipid synthesis 276
4 Lipid import into mitochondria 278
4.1 Phosphatidylserine transport 278
4.2 Phosphatidylcholine transport 280
4.3 Phosphatidylinositol transport 281
4.4 Cardiolipin precursor transport 281
4.5 Sterol transport 282
5 Lipid export from mitochondria 283
6 Concluding comments 285
References 286
From electron transfer to cholesterol transfer; molecular regulation of
steroid synthesis in the mitochondrion 293
Colin Jefcoate and Irina Artemenko 293
Abstract 293
1 Introduction 293
2 Adrenal cortex as a cholesterol processor 295
3 Adrenal mitochondrion 297
3.1 Unique functions in cholesterol transfer and regulation 297
3.2 Unusual mitochondrial morphology and its hormonal regulation .. 299
4 Mitochondrial P450 cytochromes 300
4.1 An unusual electron shuttling mechanism 300
4.2 Synthesis of mitochondrial steroidogenic enzymes 303
4.3 Can regulation of cholesterol metabolism occur at the level of
cytochrome P450scc? 305
5 The StAR protein mediates cholesterol transport 306
6 StAR function mitochondrial cholesterol metabolism 307
6.1 StAR mediates cholesterol transport 307
6.2 StAR function in releation to StAR function 307
6.3 Is StAR the key determinant of cholesterol metabolism to
pregnenolone? 309
6.4 Is mitochondrial processing of p37 StAR necessary? 310
6.5 StAR can function at the outer mitochondrial membrane 311
6.6 StAR functions from the inner mitochondrial membrane in
highly active mitochondria 313
6.7 StAR import in relation to yeast mitochondrial protein import
machinery 314
6.8 Is there an intra mitochondrial function of StAR? 316
7 Other supporting players in mitochondrial cholesterol biosynthesis 317
7.1 PBR is essential for StAR activity 317
7.2 Activation of StAR by mitochondrial free fatty acids 320
7.3 Does the form of StAR mRNA contribute to regulation of
mitochondrial uptake? 320
8 Questions unanswered 322
References 323
Index 331
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| spelling | Mitochondrial function and biogenesis with 8 tables Carla M. Koehler ... (eds.) Berlin [u.a.] Springer 2004 XVI, 333 S. Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier Topics in current genetics 8 Mitochondrium (DE-588)4124939-2 gnd rswk-swf Biogenese (DE-588)4006799-3 gnd rswk-swf (DE-588)4143413-4 Aufsatzsammlung gnd-content Mitochondrium (DE-588)4124939-2 s Biogenese (DE-588)4006799-3 s DE-604 Koehler, Carla M. Sonstige oth Topics in current genetics 8 (DE-604)BV035420113 8 HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=012824291&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
| spellingShingle | Mitochondrial function and biogenesis with 8 tables Topics in current genetics Mitochondrium (DE-588)4124939-2 gnd Biogenese (DE-588)4006799-3 gnd |
| subject_GND | (DE-588)4124939-2 (DE-588)4006799-3 (DE-588)4143413-4 |
| title | Mitochondrial function and biogenesis with 8 tables |
| title_auth | Mitochondrial function and biogenesis with 8 tables |
| title_exact_search | Mitochondrial function and biogenesis with 8 tables |
| title_full | Mitochondrial function and biogenesis with 8 tables Carla M. Koehler ... (eds.) |
| title_fullStr | Mitochondrial function and biogenesis with 8 tables Carla M. Koehler ... (eds.) |
| title_full_unstemmed | Mitochondrial function and biogenesis with 8 tables Carla M. Koehler ... (eds.) |
| title_short | Mitochondrial function and biogenesis |
| title_sort | mitochondrial function and biogenesis with 8 tables |
| title_sub | with 8 tables |
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