Kunz 1997 Mol Cell Biochem

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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.

» PMID: 9309672

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.

Keywords: Skinned muscle fibers (human), NAD(P)H fluorescence, Flavoprotein fluorescence, Mitochondrial myopathies, Encephalomyopathies - mtDNA deletions


Labels:

Stress:Mitochondrial disease  Organism: Human  Tissue;cell: Skeletal muscle 


Regulation: Redox state  Coupling state: OXPHOS 

HRR: Oxygraph-2k