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 Mitochondrial Diseases
(1994, NS 90)
Rolf Luft, Lars Ernster, Sten Orrenius
July 2-6, Grand Hotel, Saltsjöbaden
Proceedings: Molecular Basis of Disease, Biochimica et Biophysica Acta (1995), Vol 1271, no 1, Elsevier Science Ltd., Amsterdam, The Netherlands

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 successful 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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