Detection of mitochondrial defects by laser fluorimetry

Molecular and Cellular Biochemistry

Volumn 174, Issue 1-2, 1997, Pages 97-100

  Kunz, Wolfram S ^a,b   Winkler, Kirstin ^a   Kuznetsov, Andrey V ^a   Lins, Hartmut ^a   Kirches, Elmar ^a   Wallesch, Claus W ^a  

^a UNIVERSITY HOSPITAL MAGDEBURG   (Germany)

^b OTTO VON GUERICKE UNIVERSITY   (Germany)

Author keywords

Flavoprotein fluorescence; Mitochondrial myopathies and encephalomyopathies; MtDNA deletions; NAD(P)H fluorescence; Skinned muscle fibers (human)

Indexed keywords

FLAVOPROTEIN; MITOCHONDRIAL DNA; REDUCED NICOTINAMIDE ADENINE DINUCLEOTIDE PHOSPHATE; SAPONIN;

ADULT; ARTICLE; AUTOFLUORESCENCE; CASE REPORT; DELETION MUTANT; EXTERNAL OPHTHALMOPLEGIA; FEMALE; FLUORESCENCE; HUMAN; HUMAN TISSUE; LASER; MITOCHONDRIAL MYOPATHY; MITOCHONDRIAL RESPIRATION; MUSCLE BIOPSY; MUSCLE MITOCHONDRION; OXIDATION REDUCTION STATE; RESPIRATORY CHAIN; SKELETAL MUSCLE; SKINNED MUSCLE FIBER; STEADY STATE;

AGED; DNA, MITOCHONDRIAL; FEMALE; FLUOROMETRY; GENE DELETION; HUMANS; MIDDLE AGED; MITOCHONDRIA, MUSCLE; MITOCHONDRIAL MYOPATHIES; MUSCLE, SKELETAL; OPHTHALMOPLEGIA;

EID: 0030826777     PISSN: 03008177     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1006886425019     Document Type: Article

References (14)

1. Robinson BH: Lactacidemia. Biochim Biophys Acta 1182: 231-244, 1993
2. Wallace DC: Diseases of the mitochondrial DNA. Annu Rev Biochem. 61: 1175-1212, 1992
3. De Vivo DC: The expanding clinical spectrum of mitochondrial diseases. Brain and Development 15: 1-22, 1993
4. Moraes CT, DiMauro S, Zeviani M, Lombes A, Shanske S, Miranda AF, Nakase H, Bonilla E, Werneck LC, Servidei S, Nonaka I, Koga Y, Spiro AJ, Brownell AKW, Schmidt B, Schotland DL, Zupanc M, De Vivo DC, Schon EA, Rowland LP: Mitochondrial DNA deletions in progressive external ophthalmoplegia and Kearns-Sayre syndrome. N Engl J Med 320: 1293-1299, 1989
5. Moraes CT, Ciacci F, Silvestri G, Shanske S, Sciacco M, Hirano M, Schon EA, Bonilla E, DiMauro S: Atypical clinical presentations associated with the MELAS mutation at position 3243 of human mitochondrial DNA. Neuromuscul Disord 3: 43-50, 1993
6. Asn gene. Biochem Biophys Res Commun 204: 482-489, 1994
7. Kunz WS, Kuzuetsov AV, Schulze W, Eichhorn K, Schild L, Striggow F, Bohnensack R, Neuhof S, Grasshoff H, Neumann HW, Gellerich FN: Functional characterization of mitochondrial oxidative phosphorylation in saponin-skinned human muscle fibers. Biochim Biophys Acta 1144:46-53, 1993
8. Kunz WS, Kuznetsov AV, Winkler K, Gellerich FN, Neuhof S, Neumann HW: Measurement of fluorescence changes of NAD(P)H and of fluorescent flavoproteins in saponin-skinned human skeletal muscle fibers. Anal Biochem 216: 322-327, 1994
9. Haller T, Ortner M, Gnaiger E: A respirometer for investigating oxidative cell metabolism: toward optimization of respiratory studies. Anal Biochem 218: 338-342, 1994
10. Bergmeier HU: Methoden der enzymatischen Analyse. 2. Auflage, Akademie Verlag, Berlin, 1970
11. Sambrook J, Fritsch EF, Maniatis T: Molecular cloning. A laboratory manual. Cold Spring Harbor Press, New York, 1989
12. Kunz D, Luley C, Kunz WS, Winkler K, Fritz S, Bohnensack R, Wallesch CW: Oxygraphic evaluation of mitochondrial function in digitonin-permeabilized mononuclear cells and cultured skin fibroblasts of patients with chronic progressive external ophthalmoplegia. Biochem Mol Med 54: 105-111, 1995
13. Martens ME, Peterson PL, Lee CP, Nigro MA, Hart Z, Glasberg M, Hatfield JS, Chang CH: Kearns-Sayre syndrome: biochemical studies of mitochondrial metabolism. Ann Neurol 24: 630-637, 1988
14. Winkler K, Kuznetsov AV, Lins H, Kirches E, von Bossanyi P, Dietzmann K, Frank B, Feistner H, Kunz WS: Laser-excited fluorescence studies of mitochondrial function in saponin-skinned skeletal muscle fibers of patients with chronic progressive external ophthalmoplegia. Biochim Biophys Acta 1272: 181-184, 1995