Detection of Mitochondrial DiseasesF.N. Gellerich, S. Zierz In October 1995, the 1st Colloquium on Mitochondria and Myopathies in Halle/Saale was organized in Halle/Saale by the editors of this focused issue. The meeting took up what might be called an East German tradition: from 1976 to 1990 Andreas Schmidt organized seven clinically orientated Colloquia on Myology in Jena, and from 1974 to 1990 a series of twelve Colloquia on Mitochondria focused on basic research aspects was arranged by Wolfgang Kunz in Magdeburg. At those meetings, East Germany was a mediator between East European, West European and American scientists. In continuation of this tradition, scientists from more than 17 countries working on mitochondria as neurologists, biochemists, geneticists, or as physiologists came to Halle. The title of the colloquium indicated the combination of both basic and clinical mitochondrial research. The most important contributions of this meeting are now published in this focused issue. We thank all authors for their patience and cooperation that have made it possible to produce this unique collection of papers representing current knowledge on detection of mitochondrial causes of diseases. We especially thank Prof. N.S. Dhalla for making it possible to publish these contributions together in this focused issue and also as a hard-cover book. |
Contents
| 3 | |
Noninvasive quantitative ³¹P MRS assay of mitochondrial function | 17 |
F A van Dorsten T Reese J F Gellerich C J A van Echteld M G J Nederhoff H J Muller G van Vliet | 33 |
Small scale preparation of skeletal muscle mitochondria criteria of integrity and assays | 55 |
High resolution respirometry of permeabilized | 71 |
The effects of ischemia and experimental conditions on the respiration | 86 |
Oxidative phosphorylation in myocardial mitochondria in situ A calorimetric study | 101 |
Nicotinamide adenine dinucleotides permeate through mitochondrial | 115 |
Hormonal stimulation mitochondrial Ca²+ accumulation and the control of | 174 |
Nitric oxide inhibition of cytochrome oxidase and mitochondrial respiration Implications for inflammatory | 189 |
Analysis of the mitochondrial | 209 |
Regulation of mitochondrial transcription by mitochondrial | 226 |
Defects in the mitochondrial | 241 |
Adenine nucleotide translocator in dilated cardiomyopathy Pathophys | 260 |
Mitochondrial abnormalities and peripheral neuropathy in inflammatory myopathy especially | 276 |
Clinical morphological biochemical and neuroradio | 297 |
Regulation of respiration and energy transduction in cytochrome c oxidase | 131 |
Identification of mitochondrial deficiency | 149 |
The mitochondrial permeability transition in toxic hypoxic | 158 |
Ageing Effects on oxidative function of skeletal muscle in vivo 321324 | 325 |
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Common terms and phrases
1997 Kluwer Academic acid activity Address for offprints adenine nucleotide ADP concentration apoptosis assay ATP synthesis atractyloside Biochim Biophys Acta Biol Chem Ca² Ca2+ cardiac Cell Biochem 174 Cellular Biochemistry 174 chondrial clinical complex creatine kinase cyclosporin cytochrome c oxidase cytosolic decrease defects deficiency dehydrogenase deletions digitonin disease drial effect energy metabolism enzyme flux gene glutamate heart heat rate hepatocytes HK-I human increase induced inhibition inhibitor insulin intact intracellular ischemia ischemic kinetic levels LHON liver mitochondria malate measured MELAS MERRF Mi-CK mito mitochon mitochondrial DNA mitochondrial function mitochondrial membrane mitochondrial myopathy mitochondrial permeability transition MM-CK Mol Cell Biochem Molecular mtDNA mtTFA muscle mitochondria mutation NADH Neurol neuropathy normal outer membrane oxidative phosphorylation patients Physiol pore protein pyruvate rat liver ratio respiratory chain rotenone skeletal muscle studies substrates succinate syndrome synthase tion tissue tRNA uncoupling vivo