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ISSN 0925-4439 |
| Table of Contents | |
| Preface | ix |
| List of participants | xi |
| Opening session (Chair: L. Ernster) | |
| The development of mitochondrial medicine R. Luft | 1 |
| Biochemistry of mitochondrial diseases (Chair: G. F. Azzone, H. Baum) | |
| Genetic disease of
mitochondrial function evaluated by MR and NIR spectroscopy
of skeletal tissue B. Chance and W. Bank |
7 |
| Assessment of mitochondrial
function and control in normal and diseased states G. K. Radda, J. Odoom, G. Kemp, D. J. Taylor, C. Thompson and P. Styles |
15 |
| Biochemical studies of
isolated mitochondria from normal and diseased tissues C. P. Lee |
21 |
| Mitochondrial dysfunction
during anoxia and acute cell injury D. P. Jones |
29 |
| Mitochondria and oxidative stress (Chair: G. Dallner, C. P. Lee) | |
| Mitochondrial changes
associated with glutathione deficiency A. Meister |
35 |
| Influence of metabolic
inhibitors on mitochondrial permeability transition and
glutathione status D. J. Reed and M. K. Savage |
43 |
| Mitochondrial bioactivation
of cysteine S-conjugates and 4-thiaalkonoates: Implications
for mitochondrial dysfunction and mitochondrial
diseases M. W. Anders |
51 |
| The role of intracellular
oxidants in apoptosis A. F. G. Slater, C. S. I. Nobel and S. Orrenius |
59 |
| Reactive oxygen species and
the regulation of cell death by the Bc1-2 gene family S. J. Korsmeyer, X.-M. Yin, Z. N. Oltvai, D. J. Veis-Novack and G. P. Linette |
63 |
| Oxidants in mitochondria:
from physiology to diseases C. Richter, V. Gogvadze, R. Laffranchi, R. Schlapbach, M. Schweizer, M. Suter, P. Walter and M. Yaffee |
67 |
| Regulation of mitochondrial function in health and disease (Chair: H. Löw, K. Yagi) | |
| Riboflavin-responsive
complex I deficiency H. R. Scholte, H. F. M. Busch, H. D. Bakker, J. M. Bogaard, I. E. M. Luyt-Houwen and L. P. Kuyt |
75 |
| The role of thyroid hormone
and promoter diversity in the regulation of nuclear encoded
mitochondrial proteins B. D. Nelson, K. Luciakova, R. Li and S. Betina |
85 |
| Calcium ion-dependent
signalling and mitochondrial dysfunction: mitochondrial
calcium uptake during hormonal stimulation in intact liver
cells and its implication for the mitochondrial permeability
transition J. B. Hoek, J. L. Farber, A. P. Thomas and X. Wang |
93 |
| Regulation of mitochondrial
energy generation in health and disease B. Kadenbach, J. Barth, R. Akgün, R. Freund, D. Linder and S. Possekel |
103 |
| Mitochondrial antigens,
molecular mimicry and autoimmune disease H. Baum |
111 |
| Keynote lecture (Chair: S. Orrenius) | |
| Mitochondria: Beyond
oxidative phosphorylation G. Schatz |
123 |
| The mitochondrial DNA and cellular dysfunction (Chair: B. N. Ames, A. W. Linnane) | |
| Human mitochondrial
transcription factor A and promoter spacing integrity are
required for transcription initiation D. J. Dairaghi, G. S. Shadel and D. A. Clayton |
127 |
| Mitochondrial DNA (mtDNA)
disease: correlation of genotype to phenotype J. A. Morgan-Hughes, M. G. Sweeney, J. M. Cooper, S. R. Hammans, M. Brockington, A. H. V. Schapira, A. E. Harding and J. B. Clark |
135 |
| Mitochondrial DNA mutations
in human degenerative diseases and aging D. C. Wallace, J. M. Shoffner, I. Trounce, M. D. Brown, S. W. Ballinger, M. Corral-Debrinski, T. Horton, A. S. Jun and M. T. Lott |
141 |
| Searching for genes
affecting the structural integrity of the mitochondrial
genome M. Zeviani, P. Amati, G. Comi, G. Fratta, C. Mariotti and V. Tiranti |
153 |
| Role of mitochondrial DNA
and calmitine in myopathies P. Lestienne, Nelly Bataillé and B. Lucas-Heron |
159 |
| Oxidative stress and aging (Chair: D. A. Clayton, K. Folkers) | |
| Mitochondrial decay in
aging B. N. Ames, M. K. Shigenaga and T. M. Hagen |
165 |
| Modelling the effects of
age-related mtDNA mutation accumulation; Complex I
deficiency, superoxide and cell death G. Cortopassi and E. Wang |
171 |
| Mechanism of somatic
mitochondrial DNA mutations associated with age and
diseases T. Ozawa |
177 |
| The universality of
bioenergetic disease and amelioration with redox
therapy A. W. Linnane, M. Degli Esposti, M. Generowicz, A. R. Luff and P. Nagley |
191 |
| Biochemical, physiological
and medical aspects of ubiquinone function L. Ernster and G. Dallner |
195 |
| Protective roles of
cytokines against radiation: Induction of mitochondrial
MnSOD G. H. W. Wong |
205 |
| Molecular basis of mitochondrial disorders (Chair: B. D. Nelson, G. K. Radda) | |
| Deficiences of NADH and
succinate dehydrogenases in degenerative diseases and
myopathies T. P. Singer, R. R. Ramsay and B. A. C. Ackrell |
211 |
| Determination of the
structures of respiratory enzyme complexes from mammalian
mitochondria J. E. Walker |
221 |
| Paucity of deleted
mitochondrial DNAs in brain regions of Huntington's disease
patients X. Chen, E. Bonilla, M. Sciacco and E. A. Schon |
229 |
| The relationship between
the mitochondrial gene T-urf 13 and fungal pathotoxin
sensitivity in maize J. N. Siedow, D. M. Rhoads, G. C. Ward and C. S. Levings III |
235 |
| Complementation and
segregation behavior of disease-causing mitochondrial DNA
mutations in cellular model systems G. Attardi, M. Yoneda and A. Chomyn |
241 |
| Clinical aspects of mitochondrial diseases (Chair: B. Afzelius, R. Luft) | |
| Inheritance and expression
of mitochondrial DNA point mutations E. Holme, M. H. Tulinius, N.-G. Larsson, A. Oldfors |
249 |
| Mitochondrial diabetes
mellitus: a review K.-D. Gerbitz, J. M. W. van den Ouweland, J. A. Maassen and M. Jaksch |
253 |
| mtDNA mutations in Leber's
hereditary optic neuropathy M.-L. Savontaus |
261 |
| Role of mitochondria in the
etiology and pathogenesis of Parkinson's disease Y. Mizuno, S. Ikebe, N. Hattori, Y. Nakagawa-Hattori, H. Mochizuki, M. Tanaka and T. Ozawa |
265 |
| The treatment of
mitochondrial myopathies and encephalomyopathies P. L. Peterson |
275 |
| Two succesful double-blind
trials with coenzyme Q10 (Vitamin Q10)
on muscular dystrophies and neurogenic atrophies K. Folkers and R. Simonsen |
281 |
| Subject Index | 287 |
| Author Index | 291 |
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