Mitochondria
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2008
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020 | |a 0470040734 |9 0-470-04073-4 | ||
020 | |a 9780470040737 |9 978-0-470-04073-7 | ||
035 | |a (OCoLC)123692791 | ||
035 | |a (DE-599)BVBBV022876612 | ||
040 | |a DE-604 |b ger |e rakwb | ||
041 | 0 | |a eng | |
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082 | 0 | |a 571.6/57 |2 22 | |
084 | |a WE 3100 |0 (DE-625)148274: |2 rvk | ||
100 | 1 | |a Scheffler, Immo E. |e Verfasser |4 aut | |
245 | 1 | 0 | |a Mitochondria |c Immo E. Scheffler |
250 | |a 2. ed. | ||
264 | 1 | |a Hoboken, NJ |b Wiley-Liss |c 2008 | |
300 | |a XVIII, 462 S., [6] Bl. |b Ill., graph. Darst. | ||
336 | |b txt |2 rdacontent | ||
337 | |b n |2 rdamedia | ||
338 | |b nc |2 rdacarrier | ||
650 | 4 | |a Maladies mitochondriales | |
650 | 4 | |a Mitochondries | |
650 | 4 | |a DNA, Mitochondrial | |
650 | 4 | |a Genes, Mitochondrial | |
650 | 4 | |a Mitochondria | |
650 | 4 | |a Mitochondrial pathology | |
650 | 0 | 7 | |a Mitochondrium |0 (DE-588)4124939-2 |2 gnd |9 rswk-swf |
689 | 0 | 0 | |a Mitochondrium |0 (DE-588)4124939-2 |D s |
689 | 0 | |5 DE-604 | |
856 | 4 | 2 | |m Digitalisierung UB Regensburg |q application/pdf |u 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 |3 Inhaltsverzeichnis |
999 | |a oai:aleph.bib-bvb.de:BVB01-016081645 |
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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 |
any_adam_object | 1 |
any_adam_object_boolean | 1 |
author | Scheffler, Immo E. |
author_facet | Scheffler, Immo E. |
author_role | aut |
author_sort | Scheffler, Immo E. |
author_variant | i e s ie ies |
building | Verbundindex |
bvnumber | BV022876612 |
callnumber-first | Q - Science |
callnumber-label | QH603 |
callnumber-raw | QH603.M5 |
callnumber-search | QH603.M5 |
callnumber-sort | QH 3603 M5 |
callnumber-subject | QH - Natural History and Biology |
classification_rvk | WE 3100 |
ctrlnum | (OCoLC)123692791 (DE-599)BVBBV022876612 |
dewey-full | 571.6/57 |
dewey-hundreds | 500 - Natural sciences and mathematics |
dewey-ones | 571 - Physiology & related subjects |
dewey-raw | 571.6/57 |
dewey-search | 571.6/57 |
dewey-sort | 3571.6 257 |
dewey-tens | 570 - Biology |
discipline | Biologie |
discipline_str_mv | Biologie |
edition | 2. ed. |
format | Book |
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id | DE-604.BV022876612 |
illustrated | Illustrated |
index_date | 2024-07-02T18:49:10Z |
indexdate | 2024-07-09T21:07:31Z |
institution | BVB |
isbn | 0470040734 9780470040737 |
language | English |
oai_aleph_id | oai:aleph.bib-bvb.de:BVB01-016081645 |
oclc_num | 123692791 |
open_access_boolean | |
owner | DE-703 DE-355 DE-BY-UBR DE-11 DE-19 DE-BY-UBM DE-20 |
owner_facet | DE-703 DE-355 DE-BY-UBR DE-11 DE-19 DE-BY-UBM DE-20 |
physical | XVIII, 462 S., [6] Bl. Ill., graph. Darst. |
publishDate | 2008 |
publishDateSearch | 2008 |
publishDateSort | 2008 |
publisher | Wiley-Liss |
record_format | marc |
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 |
url | 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 |
work_keys_str_mv | AT schefflerimmoe mitochondria |