Effects of inhibiting CoQ 10 biosynthesis with 4-nitrobenzoate in human fibroblasts

PLoS ONE

Volumn 7, Issue 2, 2012, Pages

  Quinzii, Catarina M ^a   Tadesse, Saba ^a   Naini, Ali ^a   Hirano, Michio ^a  

^a NewYork Presbyterian Hospital   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

4 NITROBENZOIC ACID; ADENOSINE TRIPHOSPHATE; REACTIVE OXYGEN METABOLITE; UBIDECARENONE; 4-NITROBENZOIC ACID; DRUG DERIVATIVE; NITROBENZOIC ACID DERIVATIVE; UBIQUINONE;

ARTICLE; BIOENERGY; BIOSYNTHESIS; CELL COUNT; CELL DEATH; CELL HETEROGENEITY; CONTROLLED STUDY; FIBROBLAST CULTURE; HUMAN; HUMAN CELL; INHIBITION KINETICS; LIPID PEROXIDATION; LIPOPHILICITY; MITOCHONDRIAL MEMBRANE POTENTIAL; OXIDATIVE STRESS; SKIN FIBROBLAST; DOSE RESPONSE; DRUG ANTAGONISM; DRUG EFFECT; FIBROBLAST;

ADENOSINE TRIPHOSPHATE; CELL DEATH; DOSE-RESPONSE RELATIONSHIP, DRUG; FIBROBLASTS; HUMANS; NITROBENZOATES; OXIDATIVE STRESS; REACTIVE OXYGEN SPECIES; UBIQUINONE;

EID: 84857129564     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0030606     Document Type: Article

References (43)

1. Bentinger M, Tekle M, Dallner G, (2010) Coenzyme Q-biosynthesis and functions. Biochem Biophys Res Commun 396: 74-79.
2. Quinzii CM, Lopez LC, Gilkerson RW, Dorado B, Coku J, et al. (2010) Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency. FASEB J 24: 3733-3743.
3. Quinzii CM, Lopez LC, Von-Moltke J, Naini A, Krishna S, et al. (2008) Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency. FASEB J 22: 1874-1885.
4. Tran UC, Clarke CF, (2007) Endogenous synthesis of coenzyme Q in eukaryotes. Mitochondrion 7 (Suppl): S62-71.
5. Kawamukai M, (2009) Biosynthesis and bioproduction of coenzyme Q10 by yeasts and other organisms. Biotechnol Appl Biochem 53: 217-226.
6. Barros MH, Johnson A, Gin P, Marbois BN, Clarke CF, et al. (2005) The Saccharomyces cerevisiae COQ10 gene encodes a START domain protein required for function of coenzyme Q in respiration. J Biol Chem 280: 42627-42635.
7. Johnson A, Gin P, Marbois BN, Hsieh EJ, Barros MH, et al. (2005) COQ9, a new gene required for the biosynthesis of coenzyme Q in Saccharomyces cerevisiae. J Biol Chem 280: 397-404.
8. Tzagoloff A, Dieckmann CL, (1990) PET genes of Saccharomyces cerevisiae. Microbiol Rev 54: 211-225.
9. Saiki R, Nagata T, Kainou T, Matsuda H, Kawamukai M, (2005) Characterization of solanesyl and decaprenyl diphosphate synthases in mice and humans. FEBS J 272: 5606-5622.
10. Gin P, Hsu AY, Rothman SC, Jonassen T, Lee PT, et al. (2003) The Saccharomyces cerevisiae COQ6 gene encodes a mitochondrial flavin-dependent monooxygenase required for coenzyme Q biosynthesis. J Biol Chem 278: 25308-25316.
11. Do TQ, Hsu AY, Jonassen T, Lee PT, Clarke CF, (2001) A defect in coenzyme Q biosynthesis is responsible for the respiratory deficiency in Saccharomyces cerevisiae abc1 mutants. J Biol Chem 276: 18161-18168.
12. Lagier-Tourenne C, Tazir M, Lopez LC, Quinzii CM, Assoum M, et al. (2008) ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency. Am J Hum Genet 82: 661-672.
13. Mollet J, Delahodde A, Serre V, Chretien D, Schlemmer D, et al. (2008) CABC1 gene mutations cause ubiquinone deficiency with cerebellar ataxia and seizures. Am J Hum Genet 82: 623-630.
14. Tauche A, Krause-Buchholz U, Rodel G, (2008) Ubiquinone biosynthesis in Saccharomyces cerevisiae: the molecular organization of O-methylase Coq3p depends on Abc1p/Coq8p. FEMS Yeast Res 8: 1263-1275.
15. Xie LX, Hsieh EJ, Watanabe S, Allan CM, Chen JY, et al. (2011) Expression of the human atypical kinase ADCK3 rescues coenzyme Q biosynthesis and phosphorylation of Coq polypeptides in yeast coq8 mutants. Biochim Biophys Acta 1811: 348-360.
16. Padilla S, Tran UC, Jimenez-Hidalgo M, Lopez-Martin JM, Martin-Montalvo A, et al. (2009) Hydroxylation of demethoxy-Q6 constitutes a control point in yeast coenzyme Q6 biosynthesis. Cell Mol Life Sci 66: 173-186.
17. Emmanuele V, Quinzii C, Lopez LC, Berardo A, Naini A, et al. (2011) Clinical and genetic heterogeneity of coenzyme Q10 deficiency; a study of 76 patients. submitted.
18. Quinzii CM, Hirano M, (2010) Coenzyme Q and mitochondrial disease. Dev Disabil Res Rev 16: 183-188.
19. Rahman S, Clarke CF, Hirano M, (2011) 176th ENMC International Workshop: Diagnosis and treatment of coenzyme Q(10) deficiency. Neuromuscul Disord in press.
20. Quinzii C, Naini A, Salviati L, Trevisson E, Navas P, et al. (2006) A Mutation in Para-Hydroxybenzoate-Polyprenyl Transferase (COQ2) Causes Primary Coenzyme Q10 Deficiency. Am J Hum Genet 78: 345-349.
21. Lopez LC, Schuelke M, Quinzii CM, Kanki T, Rodenburg RJ, et al. (2006) Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations. Am J Hum Genet 79: 1125-1129.
22. Rodriguéz-Hernandez A, Cordero MD, Salviati L, Artuch R, Pineda M, et al. (2009) Coenzyme Q deficiency triggers mitochondrial degradation by mitophagy. Autophagy 5: 19-32.
23. Diomedi-Camassei F, Di Giandomenico S, Santorelli FM, Caridi G, Piemonte F, et al. (2007) COQ2 nephropathy: a newly described inherited mitochondriopathy with primary renal involvement. J Am Soc Nephrol 18: 2773-2780.
24. Duncan AJ, Bitner-Glindzicz M, Meunier B, Costello H, Hargreaves IP, et al. (2009) A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: a potentially treatable form of mitochondrial disease. Am J Hum Genet 84: 558-566.
25. Forsman U, Sjoberg M, Turunen M, Sindelar PJ, (2010) 4-Nitrobenzoate inhibits coenzyme Q biosynthesis in mammalian cell cultures. Nat Chem Biol 6: 515-517.
26. Lopez LC, Quinzii CM, Area E, Naini A, Rahman S, et al. (2010) Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects. PLoS One 5: e11897.
27. Robinson BH, Petrova-Benedict R, Buncic JR, Wallace DC, (1992) Nonviability of cells with oxidative defects in galactose medium: a screening test for affected patient fibroblasts. Biochem Med Metab Biol 48: 122-126.
28. Geromel V, Kadhom N, Ceballos-Picot I, Chretien D, Munnich A, et al. (2001) Human cultured skin fibroblasts survive profound inherited ubiquinone depletion. Free Radic Res 35: 11-21.
29. Lopez-Martin JM, Salviati L, Trevisson E, Montini G, DiMauro S, et al. (2007) Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis. Hum Mol Genet 16: 1091-1097.
30. Cotán D, Cordero MD, Garrido-Maraver J, Oropesa-Avila M, Rodriguez-Hernandez A, et al. (2011) Secondary coenzyme Q10 deficiency triggers mitochondria degradation by mitophagy in MELAS fibroblasts. FASEB J in press.
31. Peng M, Jarett L, Meade R, Madaio MP, Hancock WW, et al. (2004) Mutant prenyltransferase-like mitochondrial protein (PLMP) and mitochondrial abnormalities in kd/kd mice. Kidney Int 66: 20-28.
32. Falk MJ, Polyak E, Zhang Z, Peng M, King R, et al. (2011) Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice. EMBO Mol Med 3: 410-427.
33. Saiki R, Lunceford AL, Shi Y, Marbois B, King R, et al. (2008) Coenzyme Q10 supplementation rescues renal disease in Pdss2kd/kd mice with mutations in prenyl diphosphate synthase subunit 2. Am J Physiol Renal Physiol 295: F1535-1544.
34. McKenzie M, Liolitsa D, Akinshina N, Campanella M, Sisodiya S, et al. (2007) Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption. J Biol Chem 282: 36845-36852.
35. Abramov AY, Smulders-Srinivasan TK, Kirby DM, Acin-Perez R, Enriquez JA, et al. (2010) Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations. Brain 133: 797-807.
36. Busso C, Bleicher L, Ferreira-Junior JR, Barros MH, (2010) Site-directed mutagenesis and structural modeling of Coq10p indicate the presence of a tunnel for coenzyme Q6 binding. FEBS Lett 584: 1609-1614.
37. Davidson JF, Schiestl RH, (2001) Mitochondrial respiratory electron carriers are involved in oxidative stress during heat stress in Saccharomyces cerevisiae. Mol Cell Biol 21: 8483-8489.
38. Miki R, Saiki R, Ozoe Y, Kawamukai M, (2008) Comparison of a coq7 deletion mutant with other respiration-defective mutants in fission yeast. FEBS J 275: 5309-5324.
39. Uchida N, Suzuki K, Saiki R, Kainou T, Tanaka K, et al. (2000) Phenotypes of fission yeast defective in ubiquinone production due to disruption of the gene for p-hydroxybenzoate polyprenyl diphosphate transferase. J Bacteriol 182: 6933-6939.
40. Earls LR, Hacker ML, Watson JD, Miller DM 3rd, (2010) Coenzyme Q protects Caenorhabditis elegans GABA neurons from calcium-dependent degeneration. Proc Natl Acad Sci U S A 107: 14460-14465.
41. Rodriguez-Aguilera JC, Gavilan A, Asencio C, Navas P, (2005) The role of ubiquinone in Caenorhabditis elegans longevity. Ageing Res Rev 4: 41-53.
42. Nakai D, Shimizu T, Nojiri H, Uchiyama S, Koike H, et al. (2004) coq7/clk-1 regulates mitochondrial respiration and the generation of reactive oxygen species via coenzyme Q. Aging Cell 3: 273-281.
43. González-Aragón D, Burón MI, López-Lluch G, Herman MD, Gómez-Diáz C, et al. (2005) Coenzyme Q and the regulation of intracellular steady-state levels of superoxide in HL-60 cells. Biofactors 25: 31-41.