Identification of drug candidates which increase cytochrome c oxidase activity in deficient patient fibroblasts

Mitochondrion

Volumn 11, Issue 2, 2011, Pages 264-272

  Maj, Mary ^a,b   Sriskandarajah, Niroshan ^a   Hung, Vinci ^a   Browne, Ikennah ^a   Shah, Bhavank ^a   Weadge, Anita ^a   Jamieson, Nicola L ^a   Tropak, Michael ^a   Cameron, Jessie M ^a   Addis, Jane B ^a   Robinson, Brian H ^a,c  

^a HOSPITAL FOR SICK CHILDREN RESEARCH INSTITUTE   (Canada)

^b HOSPITAL FOR SICK CHILDREN   (Canada)

^c UNIVERSITY OF TORONTO   (Canada)

Author keywords

Chemical library screen; Cytochrome c oxidase; High throughput; Live cell assay; Mitochondria

Indexed keywords

BETULIN; CAPTOPRIL; CARRIER PROTEIN; CEVADINE; CYTOCHROME C OXIDASE; PEROXISOME PROLIFERATOR ACTIVATED RECEPTOR; PROTEIN LRPPRC; PROTEIN SURF1; ROSIGLITAZONE; UNCLASSIFIED DRUG; URSOLIC ACID;

ANIMAL CELL; ARTICLE; CELL ASSAY; CELL STRAIN 3T3; CONTROLLED STUDY; DRUG MECHANISM; DRUG STRUCTURE; ENZYME ACTIVITY; GENE MUTATION; HIGH THROUGHPUT SCREENING; HISTOCHEMISTRY; HUMAN; HUMAN CELL; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN ASSEMBLY; SKIN FIBROBLAST; SPECTROPHOTOMETRY; WESTERN BLOTTING;

ANIMALS; BLOTTING, WESTERN; CELL LINE, TRANSFORMED; COLORING AGENTS; ELECTRON TRANSPORT COMPLEX IV; ENZYME ACTIVATION; FIBROBLASTS; HUMANS; MICE; NIH 3T3 CELLS;

EID: 79251594110     PISSN: 15677249     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.mito.2010.10.002     Document Type: Article

References (34)

1. Bastin J., Aubey F., Rotig A., Munnich A., Djouadi F. Activation of peroxisome proliferator-activated receptor pathway stimulates the mitochondrial respiratory chain and can correct deficiencies in patients' cells lacking its components. J. Clin. Endocrinol. Metab. 2008, 93:1433-1441.
2. Burstone M.S. Histochemical localization of oxidase activity in the mitochondria of the human heart. Nature 1959, 184(Suppl 7):476-477.
3. Burstone M.S. New histochemical techniques for the demonstration of tissue oxidase (cytochrome oxidase). J. Histochem. Cytochem. 1959, 7:112-122.
4. Burstone M.S. Histochemical demonstration of cytochrome oxidase with new amine reagents. J. Histochem. Cytochem. 1960, 8:63-70.
5. Chrzanowska-Lightowlers Z.M., Turnbull D.M., Lightowlers R.N. A microtiter plate assay for cytochrome c oxidase in permeabilized whole cells. Anal. Biochem. 1993, 214:45-49.
6. De Rasmo D., Signorile A., Papa F., Roca E., Papa S. cAMP/Ca2+ response element-binding protein plays a central role in the biogenesis of respiratory chain proteins in mammalian cells. IUBMB Life 2010, 62:447-452.
7. DiMauro S. Mitochondrial diseases. Biochim. Biophys. Acta 2004, 1658:80-88.
8. Fan F., Binkowski B.F., Butler B.L., Stecha P.F., Lewis M.K., Wood K.V. Novel genetically encoded biosensors using firefly luciferase. ACS Chem. Biol. 2008, 3:346-351.
9. Feige J.N., Auwerx J. Transcriptional coregulators in the control of energy homeostasis. Trends Cell Biol. 2007, 17:292-301.
10. Glerum M., Robinson B.H., Spratt C., Wilson J., Patrick D. Abnormal kinetic behavior of cytochrome oxidase in a case of Leigh disease. Am. J. Hum. Genet. 1987, 41:584-593.
11. Glozak M.A., Sengupta N., Zhang X., Seto E. Acetylation and deacetylation of non-histone proteins. Gene 2005, 363:15-23.
12. Keilin D. Cytochromes and respiratory enzymes. Proc. R. Acad. Lond. B 1929, 104:206-252.
13. Keilin D., Hartree E.F. Cytochrome oxidase. Proc. R. Acad. Lond. B 1938, 125:171-186.
14. Kramer K.A., Oglesbee D., Hartman S.J., Huey J., Anderson B., Magera M.J., Matern D., Rinaldo P., Robinson B.H., Cameron J.M., Hahn S.H. Automated spectrophotometric analysis of mitochondrial respiratory chain complex enzyme activities in cultured skin fibroblasts. Clin. Chem. 2005, 51:2110-2116.
15. Leandro P., Gomes C.M. Protein misfolding in conformational disorders: rescue of folding defects and chemical chaperoning. Mini. Rev. Med. Chem. 2008, 8:901-911.
16. Lee C.J., Seok J.H., Hur G.M., Lee J.H., Park J.S., Seol I.C., Kim Y.H. Effects of ursolic acid, betulin and sulfur-containing compounds on mucin release from airway goblet cells. Planta Med. 2004, 70:1119-1122.
17. Liu Y., Denlinger C.E., Rundall B.K., Smith P.W., Jones D.R. Suberoylanilide hydroxamic acid induces Akt-mediated phosphorylation of p300, which promotes acetylation and transcriptional activation of RelA/p65. J. Biol. Chem. 2006, 281:31359-31368.
18. Loo T.W., Clarke D.M. Chemical and pharmacological chaperones as new therapeutic agents. Expert Rev. Mol. Med. 2007, 9:1-18.
19. Mensink M., Hesselink M.K., Russell A.P., Schaart G., Sels J.P., Schrauwen P. Improved skeletal muscle oxidative enzyme activity and restoration of PGC-1 alpha and PPAR beta/delta gene expression upon rosiglitazone treatment in obese patients with type 2 diabetes mellitus. Int. J. Obes. Lond. 2007, 31:1302-1310.
20. Miller A.D., Buttimore C. Redesign of retrovirus packaging cell lines to avoid recombination leading to helper virus production. Mol. Cell. Biol. 1986, 6:2895-2902.
21. Muceniece R., Saleniece K., Riekstina U., Krigere L., Tirzitis G., Ancans J. Betulin binds to melanocortin receptors and antagonizes alpha-melanocyte stimulating hormone induced cAMP generation in mouse melanoma cells. Cell Biochem. Funct. 2007, 25:591-596.
22. Pham N.A., Richardson T., Cameron J., Chue B., Robinson B.H. Altered mitochondrial structure and motion dynamics in living cells with energy metabolism defects revealed by real time microscope imaging. Microsc. Microanal. 2004, 10:247-260.
23. Robinson B.H., Glerum D.M., Chow W., Petrova-Benedict R., Lightowlers R., Capaldi R. The use of skin fibroblast cultures in the detection of respiratory chain defects in patients with lacticacidemia. Pediatr. Res. 1990, 28:549-555.
24. Rodgers J.T., Lerin C., Gerhart-Hines Z., Puigserver P. Metabolic adaptations through the PGC-1 alpha and SIRT1 pathways. FEBS Lett. 2008, 582:46-53.
25. Rustin P., Chretien D., Bourgeron T., Gerard B., Rotig A., Saudubray J.M., Munnich A. Biochemical and molecular investigations in respiratory chain deficiencies. Clin. Chim. Acta 1994, 228:35-51.
26. Schon E.A., DiMauro S., Hirano M., Gilkerson R.W. Therapeutic prospects for mitochondrial disease. Trends Mol. Med. 2010, 16:268-276.
27. Seimandi M., Lemaire G., Pillon A., Perrin A., Carlavan I., Voegel J.J., Vignon F., Nicolas J.C., Balaguer P. Differential responses of PPARalpha, PPARdelta, and PPARgamma reporter cell lines to selective PPAR synthetic ligands. Anal. Biochem. 2005, 344:8-15.
28. Seligman A.M., Karnovsky M.J., Wasserkrug H.L., Hanker J.S. Nondroplet ultrastructural demonstration of cytochrome oxidase activity with a polymerizing osmiophilic reagent, diaminobenzidine (DAB). J. Cell Biol. 1968, 38:1-14.
29. Smits P., Smeitink J., van den Heuvel L. Mitochondrial translation and beyond: processes implicated in combined oxidative phosphorylation deficiencies. J. Biomed. Biotechnol. 2010, 2010:1-24.
30. Stotz E., Sidwell A.E., Hogness T.R. The roll of the cytochromes in the action of "indophenol oxidase". J. Biol. Chem. 1938, 124:733-744.
31. Wagner B.K., Kitami T., Gilbert T.J., Peck D., Ramanathan A., Schreiber S.L., Golub T.R., Mootha V.K. Large-scale chemical dissection of mitochondrial function. Nat. Biotechnol. 2008, 26:343-351.
32. Wenz T., Diaz F., Spiegelman B.M., Moraes C.T. Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype. Cell Metab. 2008, 8:249-256.
33. Wilson-Fritch L., Burkart A., Bell G., Mendelson K., Leszyk J., Nicoloro S., Czech M., Corvera S. Mitochondrial biogenesis and remodeling during adipogenesis and in response to the insulin sensitizer rosiglitazone. Mol. Cell. Biol. 2003, 23:1085-1094.
34. Yang X.J., Seto E. Lysine acetylation: codified crosstalk with other posttranslational modifications. Mol. Cell 2008, 31:449-461.