High-throughput assay to measure oxygen consumption in digitonin- permeabilized cells of patients with mitochondrial disorders

Clinical Chemistry

Volumn 56, Issue 3, 2010, Pages 424-431

  Jonckheere, An I ^a   Huigsloot, Merei ^a   Janssen, Antoon J M ^a   Kappen, Antonia J H ^a   Smeitink, Jan A M ^a   Rodenburg, Richard J T ^a  

^a RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE DIPHOSPHATE; DIGITONIN; MALIC ACID; PYRUVIC ACID;

ADULT; ARTICLE; CELL MEMBRANE PERMEABILITY; CELL STIMULATION; CITRIC ACID CYCLE; COMPLEX I DEFICIENCY; CONTROLLED STUDY; CYBRID; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENERGY YIELD; ENZYME ACTIVITY; FIBROBLAST; FLUORESCENCE ANALYSIS; HIGH THROUGHPUT SCREENING; HUMAN; HUMAN CELL; HUMAN TISSUE; MITOCHONDRIAL RESPIRATION; MUSCLE MITOCHONDRION; MUSCLE TISSUE; OXIDATIVE PHOSPHORYLATION; OXYGEN CONSUMPTION; OXYGEN SENSING; VALIDITY;

CELL MEMBRANE PERMEABILITY; CELL RESPIRATION; CELLS, CULTURED; DIGITONIN; FIBROBLASTS; FLUORESCENCE; HIGH-THROUGHPUT SCREENING ASSAYS; HUMANS; MALATES; MITOCHONDRIA, MUSCLE; MITOCHONDRIAL DISEASES; OXYGEN CONSUMPTION; PYRUVIC ACID;

EID: 77649200854     PISSN: 00099147     EISSN: 15308561     Source Type: Journal    
DOI: 10.1373/clinchem.2009.131441     Document Type: Article

References (19)

1. Janssen AJ, Trijbels FJ, Sengers RC, Wintjes LT, Ruitenbeek W, Smeitink JA, et al. Measurement of the energy-generating capacity of human muscle mitochondria: diagnostic procedure and application to human pathology. Clin Chem 2006;52: 860-71.
2. Janssen AJ, Smeitink JA, van den Heuvel LP. Some practical aspects of providing a diagnostic service for respiratory chain defects. Ann Clin Biochem 2003;40:3-8.
3. Wanders RJ, Ruiter JP, Wijburg FA. Studies on mitochondrial oxidative phosphorylation in permeabilized human skin fibroblasts: application to mitochondrial encephalomyopathies. Biochim Biophys Acta 1993;1181:219-22.
4. Rizza T, Vazquez-Memije ME, Meschini MC, Bianchi M, Tozzi G, Nesti C, et al. Assaying ATP synthesis in cultured cells: a valuable tool for the diagnosis of patients with mitochondrial disorders. Biochem Biophys Res Commun 2009;383: 58-62.
5. van den Heuvel LP, Smeitink JA, Rodenburg RJ. Biochemical examination of fibroblasts in the diagnosis and research of oxidative phosphorylation (OXPHOS) defects. Mitochondrion 2004;4: 395-401.
6. Morava E, Rodenburg R, van Essen HZ, De Vries M, Smeitink J. Dietary intervention and oxidative phosphorylation capacity. J Inherit Metab Dis 2006;29:589.
7. Hynes J, Marroquin LD, Ogurtsov VI, Christiansen KN, Stevens GJ, Papkovsky DB, Will Y. Investigation of drug-induced mitochondrial toxicity using fluorescence-based oxygen-sensitive probes. Toxicol Sci 2006;92:186-200.
8. Rustin P, Chretien D, Bourgeron T, Gerard B, Rotig A, Saudubray JM, Munnich A. Biochemical and molecular investigations in respiratory chain deficiencies. Clin Chim Acta 1994;228:35-51.
9. Ferrick DA, Neilson A, Beeson C. Advances in measuring cellular bioenergetics using extracellular flux. Drug Discov Today 2008;13:268-74.
10. Will Y, Hynes J, Ogurtsov VI, Papkovsky DB. Analysis of mitochondrial function using phosphorescent oxygen-sensitive probes. Nat Protoc 2006;1:2563-72.
11. Hynes J, Hill R, Papkovsky DB. The use of a fluorescence-based oxygen uptake assay in the analysis of cytotoxicity. Toxicol In Vitro 2006;20: 785-92.
12. Bookelman H, Trijbels JM, Sengers RC, Janssen AJ, Veerkamp JH, Stadhouders AM. Pyruvate oxidation in rat and human skeletal muscle mitochondria. Biochem Med 1978;20:395-403.
13. Srere PA. Citrate synthase. Methods Enzymol 1969;13:3-11.
14. van der Westhuizen FH, van den Heuvel LP, Smeets R, Veltman JA, Pfundt R, van Kessel AG, et al. Human mitochondrial complex I deficiency: investigating transcriptional responses by microarray. Neuropediatrics 2003;34:14-22.
15. Rossignol R, Gilkerson R, Aggeler R, Yamagata K, Remington SJ, Capaldi RA. Energy substrate modulates mitochondrial structure and oxidative capacity in cancer cells. Cancer Res 2004; 64:985-93.
16. Wolf NI, Smeitink JA. Mitochondrial disorders: a proposal for consensus diagnostic criteria in infants and children. Neurology 2002;59:1402-5.
17. Taylor RW, Schaefer AM, Barron MJ, McFarland R, Turnbull DM. The diagnosis of mitochondrial muscle disease. Neuromuscul Disord 2004;14: 237-45.
18. Mracek T, Pecina P, Vojtiskova A, Kalous M, Sebesta O, Houstek J. Two components in pathogenic mechanism of mitochondrial ATPase deficiency: energy deprivation and ROS production. Exp Gerontol 2006;41:683-7.
19. Chaudhari AA, Seol JW, Kim SJ, Lee YJ, Kang HS, Kim IS, et al. Reactive oxygen species regulate Bax translocation and mitochondrial transmembrane potential, a possible mechanism for enhanced TRAIL-induced apoptosis by CCCP. Oncol Rep 2007;18:71-6.