Kunz 1997 Mol Cell Biochem: Difference between revisions
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{{Publication | {{Publication | ||
|title=Kunz WS, Winkler K, Kuznetsov AV, Lins H, Kirches E, Wallesch CW (1997) Detection of mitochondrial defects by laser fluorimetry. Mol | |title=Kunz WS, Winkler K, Kuznetsov AV, Lins H, Kirches E, Wallesch CW (1997) Detection of mitochondrial defects by laser fluorimetry. Mol Cell Biochem 174: 97-100. | ||
|info=[http://www.ncbi.nlm.nih.gov/pubmed/9309672 PMID': 9309672] | |||
|authors=Kunz WS, Winkler K, Kuznetsov AV, Lins H, Kirches E, Wallesch CW | |authors=Kunz WS, Winkler K, Kuznetsov AV, Lins H, Kirches E, Wallesch CW | ||
|year=1997 | |year=1997 | ||
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|abstract=The mitochondrial function in skeletal muscle biopsies of three patients with chronic progressive external ophthalmoplegia, having deletions of the mitochondrial DNA, was studied by laser-excited fluorescence measurements of NAD(P)H and flavoproteins in saponin-skinned fibers. We detected substantially elevated steady state redox states of the mitochondrial NAD-system in the muscle fibers of these patients. Moreover, the respiratory chain-linked autofluorescence changes in the muscle fibers of these patients were larger in comparison to controls indicating substantial alterations of the mitochondrial content. These results are in line with the presence of elevated numbers of partially respiratory chain inhibited mitochondria in the skeletal muscle of chronic progressive external ophthalmoplegia patients. | |abstract=The mitochondrial function in skeletal muscle biopsies of three patients with chronic progressive external ophthalmoplegia, having deletions of the mitochondrial DNA, was studied by laser-excited fluorescence measurements of NAD(P)H and flavoproteins in saponin-skinned fibers. We detected substantially elevated steady state redox states of the mitochondrial NAD-system in the muscle fibers of these patients. Moreover, the respiratory chain-linked autofluorescence changes in the muscle fibers of these patients were larger in comparison to controls indicating substantial alterations of the mitochondrial content. These results are in line with the presence of elevated numbers of partially respiratory chain inhibited mitochondria in the skeletal muscle of chronic progressive external ophthalmoplegia patients. | ||
|keywords=Skinned muscle fibers (human) , NAD(P)H fluorescence, Flavoprotein fluorescence, Mitochondrial myopathies, Encephalomyopathies - mtDNA deletions | |keywords=Skinned muscle fibers (human) , NAD(P)H fluorescence, Flavoprotein fluorescence, Mitochondrial myopathies, Encephalomyopathies - mtDNA deletions | ||
}} | }} | ||
{{Labeling | {{Labeling | ||
|instruments=Oxygraph-2k | |||
|injuries=Mitochondrial Disease; Degenerative Disease and Defect | |injuries=Mitochondrial Disease; Degenerative Disease and Defect | ||
|organism=Human | |organism=Human | ||
|tissues=Skeletal Muscle | |tissues=Skeletal Muscle | ||
|topics=Respiration; OXPHOS; ETS Capacity, Flux Control; Additivity; Threshold; Excess Capacity, Redox State | |topics=Respiration; OXPHOS; ETS Capacity, Flux Control; Additivity; Threshold; Excess Capacity, Redox State | ||
}} | }} |
Revision as of 12:34, 14 December 2011
Kunz WS, Winkler K, Kuznetsov AV, Lins H, Kirches E, Wallesch CW (1997) Detection of mitochondrial defects by laser fluorimetry. Mol Cell Biochem 174: 97-100. |
Kunz WS, Winkler K, Kuznetsov AV, Lins H, Kirches E, Wallesch CW (1997) Mol. Cell. Biochem.
Abstract: The mitochondrial function in skeletal muscle biopsies of three patients with chronic progressive external ophthalmoplegia, having deletions of the mitochondrial DNA, was studied by laser-excited fluorescence measurements of NAD(P)H and flavoproteins in saponin-skinned fibers. We detected substantially elevated steady state redox states of the mitochondrial NAD-system in the muscle fibers of these patients. Moreover, the respiratory chain-linked autofluorescence changes in the muscle fibers of these patients were larger in comparison to controls indicating substantial alterations of the mitochondrial content. These results are in line with the presence of elevated numbers of partially respiratory chain inhibited mitochondria in the skeletal muscle of chronic progressive external ophthalmoplegia patients. โข Keywords: Skinned muscle fibers (human), NAD(P)H fluorescence, Flavoprotein fluorescence, Mitochondrial myopathies, Encephalomyopathies - mtDNA deletions
Labels:
Stress:Mitochondrial Disease; Degenerative Disease and Defect"Mitochondrial Disease; Degenerative Disease and Defect" is not in the list (Cell death, Cryopreservation, Ischemia-reperfusion, Permeability transition, Oxidative stress;RONS, Temperature, Hypoxia, Mitochondrial disease) of allowed values for the "Stress" property. Organism: Human Tissue;cell: Skeletal Muscle"Skeletal Muscle" is not in the list (Heart, Skeletal muscle, Nervous system, Liver, Kidney, Lung;gill, Islet cell;pancreas;thymus, Endothelial;epithelial;mesothelial cell, Blood cells, Fat, ...) of allowed values for the "Tissue and cell" property.
Regulation: Respiration; OXPHOS; ETS Capacity"Respiration; OXPHOS; ETS Capacity" is not in the list (Aerobic glycolysis, ADP, ATP, ATP production, AMP, Calcium, Coupling efficiency;uncoupling, Cyt c, Flux control, Inhibitor, ...) of allowed values for the "Respiration and regulation" property., Flux Control; Additivity; Threshold; Excess Capacity"Flux Control; Additivity; Threshold; Excess Capacity" is not in the list (Aerobic glycolysis, ADP, ATP, ATP production, AMP, Calcium, Coupling efficiency;uncoupling, Cyt c, Flux control, Inhibitor, ...) of allowed values for the "Respiration and regulation" property., Redox State"Redox State" is not in the list (Aerobic glycolysis, ADP, ATP, ATP production, AMP, Calcium, Coupling efficiency;uncoupling, Cyt c, Flux control, Inhibitor, ...) of allowed values for the "Respiration and regulation" property.
HRR: Oxygraph-2k