Oxidative stress in phenylketonuria: What is the evidence?

Cellular and Molecular Neurobiology

Volumn 31, Issue 5, 2011, Pages 653-662

  Ribas, Graziela S ^a,b   Sitta, Angela ^a,b   Wajner, Moacir ^a,b   Vargas, Carmen R ^a,b  

^a FEDERAL UNIVERSITY OF RIO GRANDE DO SUL   (Brazil)

^b HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

Author keywords

Antioxidants; Free radicals; Oxidative stress; Phenylketonuria

Indexed keywords

ALPHA TOCOPHEROL; ANTIOXIDANT; ASCORBIC ACID; CATALASE; GLUTATHIONE; GLUTATHIONE DISULFIDE; GLUTATHIONE PEROXIDASE; HEME OXYGENASE 1; MALONALDEHYDE; MELATONIN; OXYGEN; PHENYLALANINE; REACTIVE NITROGEN SPECIES; REACTIVE OXYGEN METABOLITE; SELENIUM; SUPEROXIDE DISMUTASE; TETRAHYDROBIOPTERIN;

ANTIOXIDANT ACTIVITY; DIET RESTRICTION; DNA DAMAGE; ENZYME ACTIVITY; FATTY ACID OXIDATION; HUMAN; LIPID OXIDATION; NERVE DEGENERATION; NONHUMAN; OXIDATIVE STRESS; PATHOPHYSIOLOGY; PHENYLKETONURIA; PRIORITY JOURNAL; REVIEW; SUPPLEMENTATION;

ANIMALS; ANTIOXIDANTS; FREE RADICALS; HUMANS; MODELS, BIOLOGICAL; NERVE DEGENERATION; OXIDATIVE STRESS; PHENYLKETONURIAS;

ANIMALIA; RODENTIA;

EID: 79959601660     PISSN: 02724340     EISSN: 15736830     Source Type: Journal    
DOI: 10.1007/s10571-011-9693-2     Document Type: Review

References (128)

1. Acosta PB, Yannicelli S, Marriage B, Steiner R, Gaffield B, Arnold G, Lewis V, Cho S, Berstein L, Parton P, Leslie N, Korson M (1999) Protein status of infants with phenylketonuria undergoing nutrition management. J Am Coll Nutr 18:102-107 (Pubitemid 29157314)
2. Andersen JK (2004) Oxidative stress in neurodegeneration: cause or consequence? Nat Med 10(Suppl):S18-S25
3. Araujo GC, Christ SE, Steiner RD, Grange DK, Nardos B, McKinstry RC, White DA (2009) Response monitoring in children with phenylketonuria. Neuropsychology 23:130-134
4. Artuch R, Vilaseca MA, Moreno J, Lambruschini N, Cambra FJ, Campistol J (1999) Decreased serum ubiquinone-10 concentrations in phenylketonuria. Am J Clin Nutr 70:892-895 (Pubitemid 29512916)
5. Artuch R, Colomé C, Vilaseca MA, Sierra C, Cambra FJ, Lambruschini N, Campistol J (2001) Plasma phenylalanine is associated with decreased serum ubiquinone-10 concentrations in phenylketonuria. J Inherit Metab Dis 24:359-366 (Pubitemid 32677516)
6. Artuch R, Colomé C, Sierra C, Brandi N, Lambruschini N, Campistol J, Ugarte D, Vilaseca MA (2004) A longitudinal study of antioxidant status in phenylketonuric patients. Clin Biochem 37:198-203 (Pubitemid 38221567)
7. Barschak AG, Sitta A, Deon M, de Oliveira MH, Haeser A, Dutra-Filho CS, Wajner M, Vargas CR (2006) Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease. Metab Brain Dis 21:279-286 (Pubitemid 46018233)
8. Barschak AG, Sitta A, Deon M, Barden AT, Schmitt GO, Dutra-Filho CS, Wajner M, Vargas CR (2007) Erythrocyte glutathione peroxidase activity and plasma selenium concentration are reduced in maple syrup urine disease patients during treatment. Int J Dev Neurosci 25:335-338 (Pubitemid 47058895)
9. Barschak AG, Sitta A, Deon M, Barden AT, Dutra-Filho CS, Wajner M, Vargas CR (2008) Oxidative stress in plasma from maple syrup urine disease patients during treatment. Metab Brain Dis 23:71-80
10. Bauman ML, Kemper TL (1982) Morphologic and histoanatomic observations of the brain in untreated human phenylketonuria. Acta Neuropathol 58:55-63 (Pubitemid 12053178)
11. Bertelli A, Conte A, Ronca G (1994) L-propionyl carnitine protects erythrocytes and low density lipoproteins against peroxidation. Drugs Exp Clin Res 20:191-197 (Pubitemid 24352952)
12. Bindoli A, Rigobello MP, Deeble DJ (1992) Biochemical and toxicological properties of the oxidation products of catecholamines. Free Radic Biol Med 13:391-405
13. Bird S, Miller NJ, Collins JE, Rice-Evans CA (1995) Plasma antioxidant capacity in two cases of tyrosinaemia type 1: one case treated with NTBC. J Inherit Metab Dis 18:123-126
14. Boje KM, Arora PK (1992) Microglial-produced nitric oxide and reactive nitrogen oxides mediate neuronal cell death. Brain Res 587:250-256
15. Bridi R, Braun CA, Zorzi GK, Wannmacher CM, Wajner M, Lissi EG, Dutra-Filho CS (2005) Alpha-keto acids accumulating in maple syrup urine disease stimulate lipid peroxidation and reduce antioxidant defences in cerebral cortex from young rats. Metab Brain Dis 20:155-167 (Pubitemid 40768491)
16. Brigelius-Flohé R (1999) Tissue-specific functions of individual glutathione peroxidases. Free Radic Biol Med 27:951-965
17. Burri R, Steffen C, Stieger S, Brodbeck U, Colombo JP, Herschkowitz N (1990) Reduced myelinogenesis and recovery in hyperphenylalaninemic rats. Correlation between brain phenylalanine levels, characteristic brain enzymes for myelination, and brain development. Mol Chem Neuropathol 13:57-69
18. Cadenas E (1997) Basic mechanisms of antioxidant activity. Biofactors 6:391-397
19. Cadenas E, Sies H (1998) The lag phase. Free Radic Res 28:601-609 (Pubitemid 28454253)
20. Castaño A, Ayala A, Rodríguez-Gómez JA, Herrera AJ, Cano J, Machado A (1997) Low selenium diet increases the dopamine turnover in prefrontal cortex of the rat. Neurochem Int 30: 549-555 (Pubitemid 27187225)
21. Castillo M, Zafra MF, Garcia-Peregrin E (1988) Inhibition of brain and liver 3-hydroxy-3-methylglutaryl-CoA reductase and mevalonate-5-pyrophosphate decarboxylase in experimental hyperphenylalaninemia. Neurochem Res 13:551-555
22. Chinopoulos C, Adam-Vizi V (2001) Mitochondria deficient in complex I activity are depolarized by hydrogen peroxide in nerve terminals: relevance to Parkinson's disease. J Neurochem 76:302-306 (Pubitemid 32044391)
23. Colomé C, Sierra C, Antònia Vilaseca M (2000) Congenital errors of metabolism: cause of oxidative stress? Med Clin (Barc) 115: 111-117
24. Colomé C, Artuch R, Vilaseca MA, Sierra C, Brandi N, Lambruschini N, Cambra FJ, Campistol J (2003) Lipophilic antioxidants in patients with phenylketonuria. Am J Clin Nutr 77:185-188 (Pubitemid 36015390)
25. Colton C, Wilt S, Gilbert D, Chernyshev O, Snell J, Dubois-Dalcq M (1996) Species differences in the generation of reactive oxygen species by microglia. Mol Chem Neuropathol 28:15-20 (Pubitemid 26275046)
26. Costabeber E, Kessler A, Severo Dutra-Filho C, de Souza Wyse AT, Wajner M, Wannmacher CM (2003) Hyperphenylalaninemia reduces creatine kinase activity in the cerebral cortex of rats. Int J Dev Neurosci 21:111-116 (Pubitemid 36287121)
27. Curtius HC, Niederwieser A, Viscontini M, Leimbacher W, Wegmann H, Blehova B, Rey F, Schaub J, Schmidt H (1981) Serotonin and dopamine synthesis in phenylketonuria. Adv Exp Med Biol 133:277-291
28. Dalle-Donne I, Rossi R, Giustarini D, Milzani A, Colombo R (2003) Protein carbonyl groups as biomarkers of oxidative stress. Clin Chim Acta 329:23-38 (Pubitemid 36187411)
29. Dalle-Donne I, Rossi R, Colombo R, Giustarini D, Milzani A (2006) Biomarkers of oxidative damage in human disease. Clin Chem 52:601-623
30. Darling G, Mathias P, O'Regan M, Naughten E (1992) Serum selenium levels in individuals on PKU diets. J Inherit Metab Dis 15:769-773
31. de Freitas MS, de Mattos AG, Camargo MM, Wannmacher CM, Pessoa-Pureur R (1995) Effect of phenylalanine and alpha-methylphenylalanine on in vitro incorporation of 32P into cytoskeletal cerebral proteins. Neurochem Int 26:381-385
32. de Oliveira Marques F, Hagen ME, Pederzolli CD, Sgaravatti AM, Durigon K, Testa CG, Wannmacher CM, de Souza Wyse AT, Wajner M, Dutra-Filho CS (2003) Glutaric acid induces oxidative stress in brain of young rats. Brain Res 964:153-158 (Pubitemid 36165080)
33. Di Lisa F, Bernardi P (1998) Mitochondrial function as a determinant of recovery or death in cell response to injury. Mol Cell Biochem 184:379-391 (Pubitemid 28398620)
34. Dizdaroglu M, Jaruga P, Birincioglu M, Rodriguez H (2002) Free radical-induced damage to DNA: mechanisms and measurement. Free Radic Biol Med 32:1102-1115 (Pubitemid 34603348)
35. Draper HH, Hadley M (1990) A review of recent studies on the metabolism of exogenous and endogenous malondialdehyde. Xenobiotica 20:901-907 (Pubitemid 20294520)
36. Duez P, Helson M, Some TI, Dubois J, Hanocq M (2000) Chromatographic determination of 8-oxo-7, 8-dihydro-20-deoxyguanosine in cellular DNA: a validation study. Free Radic Res 33:243-260
37. El Kossi MM, Zakhary MM (2000) Oxidative stress in the context of acute cerebrovascular stroke. Stroke 31:1889-1892 (Pubitemid 30620468)
38. Ellis EM (2007) Reactive carbonyls and oxidative stress: potential for therapeutic intervention. Pharmacol Ther 115:13-24
39. Ercal N, Aykin-Burns N, Gurer-Orhan H, McDonald JD (2002) Oxidative stress in a phenylketonuria animal model. Free Radic Biol Med 32:906-911 (Pubitemid 34439261)
40. Esterbauer H, Schaur RJ, Zollner H (1991) Chemistry and biochemistry of 4-hydroxynonenal, malonaldehyde and related aldehydes. Free Radic Biol Med 11:81-128 (Pubitemid 121003917)
41. Evans P, Halliwell B (2001) Micronutrients: oxidant/antioxidant status. Br J Nutr 85(suppl 2):S67
42. Fairbairn DW, Olive PL, O'Neill KL (1995) The comet assay: a comprehensive review. Mutat Res 339:35-59
43. Fang YZ, Yang S, Wu G (2002) Free radicals, antioxidants, and nutrition. Nutrition 18:872-879 (Pubitemid 35292261)
44. Feksa LR, Cornelio AR, Rech VC, Dutra-Filho CS, Wyse AT, Wajner M, Wannmacher CM (2002) Alanine prevents the reduction of pyruvate kinase activity in brain cortex of rats subjected to chemically induced hyperphenylalaninemia. Neurochem Res 27:947-952
45. Fernandes CG, Leipnitz G, Seminotti B, Amaral AU, Zanatta A, Vargas CR, Dutra Filho CS, Wajner M (2010) Experimental evidence that phenylalanine provokes oxidative stress in hippocampus and cerebral cortex of developing rats. Cell Mol Neurobiol 30:317-326
46. Ferrari R, Ciampalini G, Agnoletti G, Cargnoni A, Ceconi C, Visioli O (1988) Effect of L-carnitine derivatives on heart mitochondrial damage induced by lipid peroxidation. Pharmacol Res Commun 20:125-132 (Pubitemid 18098919)
47. Fontella FU, Pulrolnik V, Gassen E, Wannmacher CM, Klein AB, Wajner M, Dutra-Filho CS (2000) Propionic and L-methylmalonic acids induce oxidative stress in brain of young rats. Neuroreport 11:541-544 (Pubitemid 30120473)
48. Fridovich I (1995) Superoxide radical and superoxide dismutases. Annu Rev Biochem 64:97-112
49. Fridovich I (1999) Fundamental aspects of reactive oxygen species, or what's the matter with oxygen? Ann N Y Acad Sci 893:13-18
50. Gassió R, Artuch R, Vilaseca MA, Fusté E, Colome R, Campistol J (2008) Cognitive functions and the antioxidant system in phenylketonuric patients. Neuropsychology 22:426-431 (Pubitemid 351992889)
51. Giovannini M, Verduci E, Salvatici E, Fiori L, Riva E (2007) Phenylketonuria: dietary and therapeutic challenges. J Inherit Metab Dis 30:145-152 (Pubitemid 46487994)
52. Gülçin I (2006) Antioxidant and antiradical activities of L-carnitine. Life Sci 78:803-811
53. Halliwell B (2001) Role of free radicals in the neurodegenerative diseases: therapeutic implications for antioxidant treatment. Drugs Aging 18:685-716
54. Halliwell B (2006) Oxidative stress and neurodegeneration: where are we now? J Neurochem 97:1634-1658
55. Halliwell B, Gutteridge JMC (1999) Oxidative stress: adaptation, damage, repair and death. In: Halliwell B, Gutteridge JMC (eds) Free radicals in biology and medicine. Oxford University Press, Oxford, pp 246-350
56. Halliwell B, Gutteridge JMC (2007) Free radicals in biology and medicine. Clarendon Press, Oxford
57. Hanley WB, Lee AW, Hanley AJ, Lehotay DC, Austin VJ, Schoonheyt WE, Platt BA, Clarke JT (2000) "Hypotyrosinemia" in phenylketonuria. Mol Genet Metab 69:286-294
58. Hasselbalch S, Knudsen GM, Toft PB, Hogh P, Tedeschi E, Holm S, Videbaek C, Henriksen O, Lou HC, Paulson OB (1996) Cerebral glucose metabolism is decreased in white matter changes in patients with phenylketonuria. Pediatr Res 40:21-24 (Pubitemid 26196594)
59. Hawkins CL, Morgan PE, Davies MJ (2009) Quantification of protein modification by oxidants. Free Radic Biol Med 46:965-988
60. Heales SJ, Bolaños JP, Brand MP, Clark JB, Land JM (1996) Mitochondrial damage: an important feature in a number of inborn errors of metabolism? J Inherit Metab Dis 19:140-142
61. Himwich HE (1951) Thought processes as related to brain metabolism in certain abnormal conditions. J Nerv Ment Dis 114:450-458
62. Ischiropoulos H (2003) Biological selectivity and functional aspects of protein tyrosine nitration. Biochem Biophys Res Commun 305:776-783
63. Jenner P, Olanow CW (1996) Oxidative stress and the pathogenesis of Parkinson's disease. Neurology 47:S161-S170 (Pubitemid 26422582)
64. Kaneko T, Tahara S, Matsuo M (1996) Non-linear accumulation of 8-hydroxy-20-deoxyguanosine, a marker of oxidized DNA damage, during aging. Mutat Res 316:277-285 (Pubitemid 26179270)
65. Kienzle Hagen ME, Pederzolli CD, Sgaravatti AM, Bridi R, Wajner M, Wannmacher CM, Wyse AT, Dutra-Filho CS (2002) Experimental hyperphenylalaninemia provokes oxidative stress in rat brain. Biochim Biophys Acta 1586:344-352 (Pubitemid 34457928)
66. Kovacic P, Pozos RS, Somanathan R, Shangari N, O'Brien PJ (2005) Mechanism of mitochondrial uncouplers, inhibitors, and toxins: focus on electron transfer, free radicals, and structure-activity relationships. Curr Med Chem 12:2601-2623 (Pubitemid 41460268)
67. Krause W, Halminski M, McDonald L, Dembure P, Salvo R, Freides D, Elsas L (1985) Biochemical and neuropsychological effects of elevated plasma phenylalanine in patients with treated phenylketonuria. A model for the study of phenylalanine and brain function in man. J Clin Invest 75:40-48 (Pubitemid 15163964)
68. Lambruschini N, Pérez-Dueñas B, Vilaseca MA, Mas A, Artuch R, Gassió R, Gómez L, Gutiérrez A, Campistol J (2005) Clinical and nutritional evaluation of phenylketonuric patients on tetrahydrobiopterin monotherapy. Mol Genet Metab 86(Suppl 1):S54-S60 (Pubitemid 41771110)
69. Latini A, Borba Rosa R, Scussiato K, Llesuy S, Bello-Klein A, Wajner M (2002) 3-Hydroxyglutaric acid induces oxidative stress and decreases the antioxidant defences in cerebral cortex of young rats. Brain Res 956:367-373 (Pubitemid 35351404)
70. Leipnitz G, Seminotti B, Amaral AU, de Bortoli G, Solano A, Schuck PF, Wyse AT, Wannmacher CM, Latini A, Wajner M (2008) Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats. Life Sci 82:652-662
71. Levine RL (2001) Carbonyl modified proteins in cellular regulation, aging, and disease. Free Radic Biol Med 32:790-796
72. Lissi E, Salim-Hanna M, Pascual C, del Castillo MD (1995) Evaluation of total antioxidant potential (TRAP) and total antioxidant reactivity from luminol-enhanced chemiluminescence measurements. Free Radic Biol Med 18:153-158
73. Liu D, Wen J, Liu J, Li L (1999) The roles of free radicals in amyotrophic lateral sclerosis: reactive oxygen species and elevated oxidation of protein, DNA, and membrane phospholipids. FASEB J 13:2318-2328
74. Lütz Mda G, Feksa LR, Wyse AT, Dutra-Filho CS, Wajner M, Wannmacher CM (2003) Alanine prevents the in vitro inhibition of glycolysis caused by phenylalanine in brain cortex of rats. Metab Brain Dis 18:87-94 (Pubitemid 36207339)
75. Martinez-Cruz F, Pozo D, Osuna C, Espinar A, Marchante C, Guerrero JM (2002) Oxidative stress induced by phenylketonuria in the rat: prevention by melatonin, vitamin E, and vitamin C. J Neurosci Res 69:550-558 (Pubitemid 34831415)
76. Martínez-Cruz F, Osuna C, Guerrero JM (2006) Mitochondrial damage induced by fetal hyperphenylalaninemia in the rat brain and liver: its prevention by melatonin, Vitamin E, and Vitamin C. Neurosci Lett 392:1-4 (Pubitemid 41757565)
77. Masella R, Di Benedetto R, Varì R, Filesi C, Giovannini C (2005) Novel mechanisms of natural antioxidant compounds in biological systems: involvement of glutathione and glutathione-related enzymes. J Nutr Biochem 16:577-586 (Pubitemid 41338369)
78. McCord JM (2000) The evolution of free radicals and oxidative stress. Am J Med 108:652-659
79. Miller DM, Buettner GR, Aust SD (1990) Transition metals as catalysts of "autoxidation" reactions. Free Radic Biol Med 8:95-108
80. Moncada S, Higgs A (1993) The L-arginine-nitric oxide pathway. N Engl J Med 329:2002-2012 (Pubitemid 24015045)
81. Moraes TB, Zanin F, da Rosa A, de Oliveira A, Coelho J, Petrillo F, Wajner M, Dutra-Filho CS (2010) Lipoic acid prevents oxidative stress in vitro and in vivo by an acute hyperphenylalaninemia chemically-induced in rat brain. J Neurol Sci 292:89-95
82. Moyano D, Vilaseca MA, Pineda M, Campistol J, Vernet A, Póo P, Artuch R, Sierra C (1997) Tocopherol in inborn errors of intermediary metabolism. Clin Chim Acta 263:147-155 (Pubitemid 27285752)
83. Nigam MP, Labar DR (1979) The effect of hyperphenylalaninemia on size and density of synapses in rat neocortex. Brain Res 179:195-198 (Pubitemid 10163701)
84. Niki E (2009) Lipid peroxidation: physiological levels and dual biological effects. Free Radic Biol Med 47:469-484
85. Perry G, Taddeo MA, Petersen RB, Castellani RJ, Harris PL, Siedlak SL, Cash AD, Liu Q, Nunomura A, Atwood CS, Smith MA (2003) Adventiously-bound redox active iron and copper are at the center of oxidative damage in Alzheimer disease. Biometals 16:77-81 (Pubitemid 36140217)
86. Pietz J (1998) Neurological aspects of adult phenylketonuria. Curr Opin Neurol 11:679-688
87. Poustie VJ, Wildgoose J (2010) Dietary interventions for phenylketonuria. Cochrane Database Syst Rev 20:CD001304
88. Przyrembel H, Bremer HJ (2000) Nutrition, physical growth, and bone density in treated phenylketonuria. Eur J Pediatr 159(Suppl 2):S129-S135
89. Rech VC, Feksa LR, Dutra-Filho CS, Wyse AT, Wajner M, Wannmacher CM (2002) Inhibition of the mitochondrial respiratory chain by phenylalanine in rat cerebral cortex. Neurochem Res 27:353-357 (Pubitemid 34579340)
90. Reilly C, Barrett JE, Patterson CM, Tinggi U, Latham SL, Marrinan A (1990) Trace element nutrition status and dietary intake of children with phenylketonuria. Am J Clin Nutr 52:159-165
91. Reynolds A, Laurie C, Mosley RL, Gendelman HE (2007) Oxidative stress and the pathogenesis of neurodegenerative disorders. Int Rev Neurobiol 82:297-325
92. Reznick AZ, Packer L (1993) Free radicals and antioxidants in muscular neurological diseases and disorders. In: Poli G, Albano E, Dianzani MU (eds) Free radicals: from basic science to medicine. Birkhäuser Verlag, Basel, pp 425-437
93. Ribas GS, Manfredini V, de Mari JF, Wayhs CY, Vanzin CS, Biancini GB, Sitta A, Deon M, Wajner M, Vargas CR (2010) Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of L-carnitine supplementation. Int J Dev Neurosci 28:127-132
94. Richard E, Alvarez-Barrientos A, Pérez B, Desviat LR, Ugarte M (2007) Methylmalonic acidaemia leads to increased production of reactive oxygen species and induction of apoptosis through the mitochondrial/caspase pathway. J Pathol 213:453-461 (Pubitemid 350206516)
95. Richter C (1987) Biophysical consequences of lipid peroxidation in membranes. Chem Phys Lipids 44:175-189
96. Rodrigues NR, Wannmacher CM, Dutra-Filho CS, Pires RF, Fagan PR, Wajner M (1990) Effect of phenylalanine, p-chlorophenylalanine and alpha- methylphenylalanine on glucose uptake in vitro by the brain of young rats. Biochem Soc Trans 18:419 (Pubitemid 20181056)
97. Rottoli A, Lista G, Zecchini G, Butté C, Longhi R (1985) Plasma selenium levels in treated phenylketonuric patients. J Inherit Metab Dis Suppl 2:127-128 (Pubitemid 16227165)
98. Schulpis KH, Nounopoulos C, Scarpalezou A, Bouloukos A, Missiou- Tsagarakis S (1990) Serum carnitine level in phenylketonuric children under dietary control in Greece. Acta Paediatr Scand 79:930-934 (Pubitemid 20333500)
99. Schulpis KH, Tsakiris S, Karikas GA, Moukas M, Behrakis P (2003) Effect of diet on plasma total antioxidant status in phenylketonuric patients. Eur J Clin Nutr 57:383-387 (Pubitemid 36240495)
100. Schulpis KH, Tsakiris S, Traeger-Synodinos J, Papassotiriou I (2005) Low total antioxidant status is implicated with high 8-hydroxy- 2-deoxyguanosine serum concentrations in phenylketonuria. Clin Biochem 38:239-242 (Pubitemid 40222323)
101. Scriver CR, Kaufmann S (2001) The hyperphenylalaninemias. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic and molecular basis of inherited disease. McGraw-Hill, New York, pp 1667-1724
102. Shah SN, Peterson NA, McKean CM (1972) Lipid composition of human cerebral white matter and myelin in phenylketonuria. J Neurochem 19:2369-2376
103. Sierra C, Vilaseca MA, Moyano D, Brandi N, Campistol J, Lambruschini N, Cambra FJ, Deulofeu R, Mira A (1998) Antioxidant status in hyperphenylalaninemia. Clin Chim Acta 276:1-9 (Pubitemid 28386824)
104. Sies H (1985) Oxidative stress: introductory remarks. In: Sies H (ed) Oxidative stress. Academic Press, London, pp 1-7
105. Sirtori LR, Dutra-Filho CS, Fitarelli D, Sitta A, Haeser A, Barschak AG, Wajner M, Coelho DM, Llesuy S, Belló-Klein A, Giugliani R, Deon M, Vargas CR (2005) Oxidative stress in patients with phenylketonuria. Biochim Biophys Acta 1740:68-73 (Pubitemid 40692700)
106. Sitta A, Barschak AG, Deon M, Terroso T, Pires R, Giugliani R, Dutra-Filho CS, Wajner M, Vargas CR (2006) Investigation of oxidative stress parameters in treated phenylketonuric patients. Metab Brain Dis 21:287-296 (Pubitemid 46018234)
107. Sitta A, Manfredini V, Biasi L, Treméa R, Schwartz IV, Wajner M, Vargas CR (2009a) Evidence that DNA damage is associated to phenylalanine blood levels in leukocytes from phenylketonuric patients. Mutat Res 679:13-16
108. Sitta A, Barschak AG, Deon M, de Mari JF, Barden AT, Vanzin CS, Biancini GB, Schwartz IV, Wajner M, Vargas CR (2009b) L-carnitine blood levels and oxidative stress in treated phenylketonuric patients. Cell Mol Neurobiol 29:211-218
109. Sitta A, Barschak AG, Deon M, Barden AT, Biancini GB, Vargas PR, de Souza CF, Netto C, Wajner M, Vargas CR (2009c) Effect of short- and long-term exposition to high phenylalanine blood levels on oxidative damage in phenylketonuric patients. Int J Dev Neurosci 27:243-247
110. Smith I, Knowles J (2000) Behaviour in early treated phenylketonuria: a systematic review. Eur J Pediatr 159(Suppl 2):S89-S93
111. Solano AF, Leipnitz G, De Bortoli GM, Seminotti B, Amaral AU, Fernandes CG, Latini AS, Dutra-Filho CS, Wajner M (2008) Induction of oxidative stress by the metabolites accumulating in isovaleric acidemia in brain cortex of young rats. Free Radic Res 42:707-715
112. Steiner G, Menzel H, Lombeck I, Ohnesorge FK, Bremer HJ (1982) Plasma glutathione peroxidase after selenium supplementation in patients with reduced selenium state. Eur J Pediatr 138:138-140 (Pubitemid 12141766)
113. +- ATPase activity induced by homocysteine is probably mediated by oxidative stress. Neurochem Res 26:1195-1200
114. Streck EL, Vieira PS, Wannmacher CM, Dutra-Filho CS, Wajner M, Wyse AT (2003) In vitro effect of homocysteine on some parameters of oxidative stress in rat hippocampus. Metab Brain Dis 18:147-154
115. Teunissen CE, de Vente J, Steinbusch HW, De Bruijn C (2002) Biochemical markers related to Alzheimer's dementia in serum and cerebrospinal fluid. Neurobiol Aging 23:485-508 (Pubitemid 34495461)
116. Uchida K (2003) 4-Hydroxy-2-nonenal: a product and mediator of oxidative stress. Prog Lipid Res 42:318-343
117. Ushakova GA, Gubkina HA, Kachur VA, Lepekhin EA (1997) Effect of experimental hyperphenylalaninemia on the postnatal rat brain. Int J Dev Neurosci 15:29-36 (Pubitemid 27135433)
118. Valko M, Izakovic M, Mazur M, Rhodes CJ, Telser J (2004) Role of oxygen radicals in DNA damage and cancer incidence. Mol Cell Biochem 266:37-56 (Pubitemid 39562306)
119. Valko M, Leibfritz D, Moncol J, Cronin MT, Mazur M, Telser J (2007) Free radicals and antioxidants in normal physiological functions and human disease. Int J Biochem Cell Biol 39:44-84 (Pubitemid 44566469)
120. van Bakel MM, Printzen G, Wermuth B, Wiesmann UN (2000) Antioxidant and thyroid hormone status in selenium-deficient phenylketonuric and hyperphenylalaninemic patients. Am J Clin Nutr 72:976-981
121. Wajner M, Latini A, Wyse AT, Dutra-Filho CS (2004) The role of oxidative damage in the neuropathology of organic acidurias: insights from animal studies. J Inherit Metab Dis 27:427-448 (Pubitemid 39140134)
122. Weber C, Bysted A, Holmer G (1997) Coenzyme Q10 in the dietdaily intake and relative bioavailability. Mol Aspects Med 18(Suppl):S251-S254
123. Weglage J, Pietsch M, Fünders B, Koch HG, Ullrich K (1995) Neurological findings in early treated phenylketonuria. Acta Paediatr 84:411-415
124. Wilke BC, Vidailhet M, Favier A, Guillemin C, Ducros V, Arnaud J, Richard MJ (1992) Selenium, glutathione peroxidase (GSH-Px) and lipid peroxidation products before and after selenium supplementation. Clin Chim Acta 207:137-142
125. +, K(+)-ATPase in synaptic plasma membranes from cerebral cortex of rats. Biochem Soc Trans 23:227S
126. +-ATPase activity in experimental phenylketonuria. Metab Brain Dis 14:95-101
127. Wyse AT, Zugno AI, Streck EL, Matté C, Calcagnotto T, Wannmacher CM, Wajner M (2002) Inhibition of Na(+), K(+)-ATPase activity in hippocampus of rats subjected to acute administration of homocysteine is prevented by vitamins E and C treatment. Neurochem Res 27:1685-1689 (Pubitemid 36020123)
128. Zheng M, Storz G (2000) Redox sensing by prokaryotic transcription factors. Biochem Pharmacol 59:1-6