Primary and secondary drug screening assays for Friedreich ataxia

Journal of Biomolecular Screening

Volumn 17, Issue 3, 2012, Pages 303-313

  Cotticelli, M Grazia ^a   Rasmussen, Lynn ^b   Kushner, Nicole L ^b   McKellip, Sara ^b   Sosa, Melinda Ingrum ^b   Manouvakhova, Anna ^b   Feng, Shuang ^b   White, E Lucile ^b   Maddry, Joseph A ^b   Heemskerk, Jill ^c   Oldt, Robert J ^a   Surrey, Lea F ^a   Ochs, Rachel ^a   Wilson, Robert B ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b SOUTHERN RESEARCH INSTITUTE   (United States)

^c NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE   (United States)

Author keywords

Friedreich ataxia; high throughput screening; WST 1; yeast; Yfh1p

Indexed keywords

2,2 BISEPIGALLOCATECHIN MONOGALLATE; ADENOSINE TRIPHOSPHATE; EPIGALLOCATECHIN 3,5 DIGALLATE; FRATAXIN; IDEBENONE; IRON; KATACINE; MENADIONE; PARAROSANILINE EMBONATE; PYRVINIUM EMBONATE; RIBOFLAVIN DERIVATIVE; SENNOSIDE B; TANNIN; THEAFLAVIN MONGALLATE; THEAFLAVINE DIGALLATE; UNCLASSIFIED DRUG;

ANIMAL CELL; ARTICLE; CARBON SOURCE; CELL VIABILITY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; DRUG SCREENING; ELECTRON TRANSPORT; FRIEDREICH ATAXIA; HIGH THROUGHPUT SCREENING; IN VITRO STUDY; MAMMAL CELL; MITOCHONDRION; MOUSE; NONHUMAN; OXIDATION REDUCTION POTENTIAL; OXIDATIVE STRESS; PRIORITY JOURNAL;

ANIMALS; CELL LINE; DRUG EVALUATION, PRECLINICAL; FRIEDREICH ATAXIA; HIGH-THROUGHPUT SCREENING ASSAYS; IRON-BINDING PROTEINS; IRON-SULFUR PROTEINS; MICE; MITOCHONDRIA; MITOCHONDRIAL DISEASES; OXIDATIVE STRESS; SACCHAROMYCES CEREVISIAE; TETRAZOLIUM SALTS;

MAMMALIA; MURINAE;

EID: 84859030208     PISSN: 10870571     EISSN: 1552454X     Source Type: Journal    
DOI: 10.1177/1087057111427949     Document Type: Article

References (27)

1. Pandolfo, M. Friedreich Ataxia. Arch. Neurol. 2008, 65, 1296-1303.
2. Durr, A.; Cossee, M.; Agid, Y.; Campuzano, V.; Mignard, C.; Penet, C.; Mandel, J. L.; Brice, A.; Koenig, M. Clinical and Genetic Abnormalities in Patients with Friedreich's Ataxia. N. Engl. J. Med. 1996, 335, 1169-1175. (Pubitemid 26339770)
3. Campuzano, V.; Montermini, L.; Moltò, M. D.; Pianese, L.; Cossee, M.; Cavalcanti, F.; Monros, E.; Rodius, F.; Duclos, F.; Monticelli, A.; et al. Friedreich's Ataxia: Autosomal Recessive Disease Caused by an Intronic GAA Triplet Repeat Expansion [see comments]. Science 1996, 271, 1423-1427. (Pubitemid 26089479)
4. Muhlenhoff, U.; Richhardt, N.; Ristow, M.; Kispal, G.; Lill, R. The Yeast Frataxin Homolog Yfh1p Plays a Specific Role in the Maturation of Cellular Fe/S Proteins. Hum. Mol. Genet. 2002, 11, 2025-2036. (Pubitemid 34919277)
5. Muhlenhoff, U.; Gerber, J.; Richhardt, N.; Lill, R. Components Involved in Assembly and Dislocation of Iron-Sulfur Clusters on the Scaffold Protein Isu1p. Embo. J. 2003, 22, 4815-4825. (Pubitemid 37162917)
6. Babcock, M.; de Silva, D.; Oaks, R.; Davis-Kaplan, S.; Jiralerspong, S.; Montermini, L.; Pandolfo, M.; Kaplan, J. Regulation of Mitochondrial Iron Accumulation by Yfh1p, a Putative Homolog of Frataxin. Science 1997, 276, 1709-1712. (Pubitemid 27421980)
7. Wilson, R. B.; Roof, D. M. Respiratory Deficiency Due toLoss of Mitochondrial DNA in Yeast Lacking the Frataxin Homologue. Nat. Genet. 1997, 16, 352-357. (Pubitemid 27323299)
8. Foury, F.; Cazzalini, O. Deletion of the Yeast Homologue of the Human Gene Associated with Friedreich's Ataxia Elicits Iron Accumulation in Mitochondria. FEBS Lett. 1997, 411, 373-377. (Pubitemid 27301488)
9. Radisky, D. C.; Babcock, M. C.; Kaplan, J. K. The Yeast Frataxin Homologue Mediates Mitochondrial Iron Efflux: Evidence for a Mitochondrial Iron Cycle. J. Biol. Chem. 1999, 274, 4497-4499.
10. Foury, F. Low Iron Concentration and Aconitase Deficiency in a Yeast Frataxin Homologue Deficient Strain. FEBS Lett. 1999, 456, 281-284. (Pubitemid 29352308)
11. Rotig, A.; de Lonlay, P.; Chretien, D.; Foury, F.; Koenig, M.; Sidi, D.; Munnich, A.; Rustin, P. Aconitase and Mitochondrial Iron-Sulphur Protein Deficiency in Friedreich's Ataxia. Nat. Genet. 1997, 17, 215-217.
12. Bradley, J. L.; Blake, J. C.; Chamberlain, S.; Thomas, P. K.; Cooper, J. M.; Schapira, A. H. Clinical, Biochemical and Molecular Genetic Correlations in Friedreich's Ataxia. Hum. Mol. Genet. 2000, 9, 275-282. (Pubitemid 30052751)
13. Lodi, R.; Cooper, J. M.; Bradley, J. L.; Manners, D.; Styles, P.; Taylor, D. J.; Schapira, A. H. Deficit of In Vivo Mitochondrial ATP Production in Patients with Friedreich Ataxia. Proc. Nat. Acad. Sci. U. S. A. 1999, 96, 11492-11495. (Pubitemid 29487408)
14. Lodi, R.; Hart, P. E.; Rajagopalan, B.; Taylor, D. J.; Crilley, J. G.; Bradley, J. L.; Blamire, A. M.; Manners, D.; Styles, P.; Schapira, A. H.; et al. Antioxidant Treatment Improves In Vivo Cardiac and Skeletal Muscle Bioenergetics in Patients with Friedreich's Ataxia. Ann. Neurol. 2001, 49, 590-596.
15. Puccio, H.; Simon, D.; Cossee, M.; Criqui-Filipe, P.; Tiziano, F.; Melki, J.; Hindelang, C.; Matyas, R.; Rustin, P.; Koenig, M. Mouse Models for Friedreich Ataxia Exhibit Cardiomyopathy, Sensory Nerve Defect and Fe-S Enzyme Deficiency Followed by Intramitochondrial Iron Deposits. Nat. Genet. 2001, 27, 181-186. (Pubitemid 32157445)
16. Lynch, D. R.; Lech, G.; Farmer, J. M.; Balcer, L. J.; Bank, W.; Chance, B.; Wilson, R. B. Near-Infrared Muscle Spectroscopy in Patients with Friedreich's Ataxia. Muscle Nerve 2002, 25, 664-673. (Pubitemid 34462605)
17. Schulz, J. B.; Dehmer, T.; Schols, L.; Mende, H.; Hardt, C.; Vorgerd, M.; Biirk, K.; Dichgans, J.; Beal, M. F. Oxidative Stress in Patients with Friedreich Ataxia [see comments]. Neurology 2000, 55, 1719-1721.
18. Emond, M.; Lepage, G.; Vanasse, M.; Pandolfo, M. Increased Levels of Plasma Malondialdehyde in Friedreich Ataxia [see comments]. Neurology 2000, 55, 1752-1753.
19. Beecher, G. R. Overview of Dietary Flavonoids: Nomenclature, Occurrence and Intake. J. Nutr. 2003, 133, 3248S-3254S. (Pubitemid 37222252)
20. Manach, C.; Scalbert, A.; Morand, C.; Remesy, C.; Jimenez, L. Polyphenols: Food Sources and Bioavailability. Am. J. Clin. Nutr. 2004, 79, 727-747. (Pubitemid 41103873)
21. Mandel, S.; Amit, T.; Reznichenko, L.; Weinreb, O.; Youdim, M. B. Green Tea Catechins asBrain-Permeable, Natural Iron Chelators - Antioxidants for the Treatment of Neurodegenerative Disorders. Mol. Nutr. Food Res. 2006, 50, 229-234. (Pubitemid 43325663)
22. Guo, Q.; Zhao, B.; Li, M.; Shen, S.; Xin, W. Studies onProtective Mechanisms of Four Components of Green Tea Polyphenols against Lipid Peroxidation in Synaptosomes. Biochim. Biophys. Acta 1996, 1304, 210-222.
23. Kumamoto, M.; Sonda, T.; Nagayama, K.; Tabata, M. Effects of pH and Metal Ions on Antioxidative Activities of Catechins. Biosci. Biotechnol. Biochem. 2001, 65, 126-132. (Pubitemid 33617450)
24. Hausse, A. O.; Aggoun, Y.; Bonnet, D.; Sidi, D.; Munnich, A.; Rotig, A.; Rustin, P. Idebenone and Reduced Cardiac Hypertrophy in Friedreich's Ataxia [see comments]. Heart 2002, 87, 346-349. (Pubitemid 34270947)
25. Mariotti, C.; Solari, A.; Torta, D.; Marano, L.; Fiorentini, C.; Di Donato, S. Idebenone Treatment in Friedreich Patients: One-Year-Long Randomized Placebo-Controlled Trial. Neurology 2003, 60, 1676-1679. (Pubitemid 36618100)
26. Wilson, R. B. Experimental Therapeutics for Friedreich Ataxia. In Genetic Instabilities and Neurological Diseases; Wells, R. D., and Ashizawa, T.; Eds. Elsevier: Burlington, MA, 2006; pp 297-303.
27. Di Prospero, N. A.; Sumner, C. J.; Penzak, S. R.; Ravina, B.; Fischbeck, K. H.; Taylor, J. P. Safety, Tolerability, and Pharmacokinetics of High-Dose Idebenone in Patients with Friedreich Ataxia. Arch. Neurol. 2007, 64, 803-808. (Pubitemid 46919834)