Mitochondrial Diseases: Models and Methods

Front Matter....Pages I-XI
Introduction....Pages 1-4
Organization and Expression of the Mitochondrial Genome....Pages 5-15
Mitochondrial DNA Inheritance in Mammals....Pages 17-31
Molecular Basis of Mitochondrial DNA Diseases....Pages 33-58
Structural Studies on Human Mitochondrial tRNAs....Pages 59-72
Structure, Function and Pathology of Complex I....Pages 73-86
Complex II or Succinate: Quinone Oxidoreductase and Pathology....Pages 87-95
The bc1 Complex in the Mitochondrial Respiratory Chain....Pages 97-113
Cytochrome c Oxidase and Mitochondrial Pathology....Pages 115-127
ATPase-ATP Synthase and Mitochondrial Pathology....Pages 129-142
Physiology and Pathophysiology of the Mitochondrial ADP/ATP Carrier....Pages 143-158
The Normal and Pathological Structure, Function and Expression of Mitochondrial Creatine Kinase....Pages 159-172
Pyruvate Dehydrogenase Deficiencies....Pages 173-184
Electrochemical Gradient and Mitochondrial DNA in Living Cells....Pages 185-196
A Heteroplasmic Strain of D. Subobscura . An Animal Model of Mitochondrial Genome Rearrangement....Pages 197-208
Stability of the Mitochondrial Genome of Podospora anserina and Its Genetic Control....Pages 209-228
The Control of Ageing and Mitochondria....Pages 229-238
Roles of Mitochondria in Apoptosis....Pages 239-254
The Triiodothyronine Mitochondrial Pathway....Pages 255-270
Cytoskeleton - Mitochondrial Interactions....Pages 271-281
NADH Brain Determination by Micromeasurements of a Laser-Induced Fluorescence in the Rat....Pages 283-294
Allotopic Expression of Mitochondrial Genes: Further Steps in the Evolution of Eukaryotic Cells and Therapeutic Strategy....Pages 295-302
Import of tRNA into Yeast Mitochondria: Experimental Approaches and Possible Applications....Pages 303-316
The Import of Cytosolic tRNA into Plant Mitochondria....Pages 317-326
Plant Cytoplasmic Male Sterility: A Mitochondrial Pathology and Its Biotechnological Application....Pages 327-341
Histopathology of Skeletal Muscle Mitochondria....Pages 343-355
Enzymatic and Polarographic Measurements of the Respiratory Chain Complexes....Pages 357-377
Mitochondrial DNA Analysis....Pages 379-387
Back Matter....Pages 389-396