Evidence that l-carnitine and selenium supplementation reduces oxidative stress in phenylketonuric patients

Cellular and Molecular Neurobiology

Volumn 31, Issue 3, 2011, Pages 429-436

  Sitta, A ^a,b   Vanzin, C S ^a,b   Biancini, G B ^a,b   Manfredini, V ^b,c   De Oliveira, A B ^a   Wayhs, C A Y ^a,b   Ribas, G O S ^a,b   Giugliani, L ^a   Schwartz, I V D ^a   Bohrer, D ^d   Garcia, S C ^b   Wajner, M ^a,b   Vargas, C R ^a,b  

^a HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

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

^c DEPARTMENT OF FOOD ENGINEERING   (Brazil)

^d FEDERAL UNIVERSITY OF SANTA MARIA   (Brazil)

Author keywords

l Carnitine; Oxidative stress; Phenylketonuria; Selenium

Indexed keywords

CARNITINE; GLUTATHIONE PEROXIDASE; SELENIUM;

ADOLESCENT; ADULT; ARTICLE; BLOOD LEVEL; CLINICAL ARTICLE; CONTROLLED STUDY; DIET SUPPLEMENTATION; ENZYME ACTIVITY; HUMAN; HUMAN CELL; LIPID PEROXIDATION; OXIDATIVE STRESS; PHENYLKETONURIA; PRIORITY JOURNAL; PROTEIN RESTRICTION; SULFOXIDATION;

ADOLESCENT; ANTIOXIDANTS; CARNITINE; DIETARY SUPPLEMENTS; HUMANS; OXIDATIVE STRESS; PHENYLKETONURIAS; REACTIVE OXYGEN SPECIES; SELENIUM; THIOBARBITURIC ACID REACTIVE SUBSTANCES; YOUNG ADULT;

EID: 79955563188     PISSN: 02724340     EISSN: None     Source Type: Journal    
DOI: 10.1007/s10571-010-9636-3     Document Type: Article

References (50)

1. PB Acosta 1996 Nutrition studies in treated infants and children with phenylketonuria: vitamins, minerals, trace elements Eur J Pediatr 155 136 139 10.1007/PL00014231
2. RM Adibhatla JF Hatcher 2010 Lipid oxidation and peroxidation in CNS health and disease: from molecular mechanisms to therapeutic opportunities Antioxid Redox Signal 12 125 169 10.1089/ars.2009.2668 1:CAS:528: DC%2BC3cXhsFejt7k%3D 19624272
3. H Aebi 1984 Catalase in vitro Methods Enzymol 105 121 126 10.1016/S0076-6879(84)05016-3 1:CAS:528:DyaL2cXltVKis7s%3D 6727660
4. MY Aksenov WR Markesbery 2001 Changes in thiol content and expression of glutathione redox system genes in the hippocampus and cerebellum in Alzheimer's disease Neurosci Lett 302 141 145 10.1016/S0304-3940(01)01636-6 1:CAS:528:DC%2BD3MXisVWnsrs%3D 11290407 (Pubitemid 32274229)
5. R Artuch MA Vilaseca J Moreno N Lambruschini FJ Cambra J Campistol 1999 Decreased serum ubiquinone-10 concentration in phenylketonuria Am J Clin Nutr 70 892 895 1:CAS:528:DyaK1MXntFCgurY%3D 10539751 (Pubitemid 29512916)
6. R Artuch C Colome MA Vilaseca C Sierra FJ Cambra N Lambruschini J Campistol 2001 Plasma phenylalanine is associated with decreased serum ubiquinone-10 concentrations in phenylketonuria J Inherit Metab Dis 24 359 366 10.1023/A:1010500502275 1:CAS:528:DC%2BD3MXmtVOgurc%3D 11486901 (Pubitemid 32677516)
7. R Artuch C Colome C Sierra N Brandi N Lambruschini J Campistol D Ugarte MA Vilaseca 2004 A longitudinal study of antioxidant status in phenylketonuric patients Clin Biochem 37 198 203 10.1016/j.clinbiochem.2003.10.017 1:CAS:528:DC%2BD2cXht1yqsL0%3D 14972641 (Pubitemid 38221567)
8. AG Barschak A Sitta M Deon EN Busanello DM Coelho F Cipriani CS Dutra-Filho R Giugliani M Wanjer CR Vargas 2009 Amino acids levels and lipid peroxidation in maple syrup urine disease patients Clin Biochem 42 462 466 10.1016/j.clinbiochem.2008.12.005 1:CAS:528:DC%2BD1MXjtF2qtLs%3D 19121297
9. H Bickel J Gerrard EM Hickmans 1953 Influence of phenylalanine intake on phenylketonuria Lancet 265 812 813 10.1016/S0140-6736(53)90473-5 1:CAS:528:DyaG2MXhslSitQ%3D%3D 13098090
10. F Carmagnol PM Sinet H Jerome 1983 Selenium-dependent and nonselenium-dependent glutathione peroxidases in human tissue extracts Biochim Biophys Acta 759 49 57 1:CAS:528:DyaL3sXltlGqtLs%3D 6882790 (Pubitemid 13013120)
11. DH Chace SL Hillman JLK Van Hove EW Naylor 1997 Rapid diagnosis of MCAD deficiency: quantitative analysis of octanoylcarnitine and other acylcarnitines in newborn blood spots by tandem mass spectrometry Clin Chem 43 2106 2113 1:CAS:528:DyaK2sXnt1ejsL0%3D 9365395 (Pubitemid 27497809)
12. Chaudière J (1994) Some chemical and biochemical constraints of oxidative stress in living cells. In: Riee-Evans CA, Burdon RH (eds) Free radical damage and its control. Elsevier, Amsterdam, pp 25-66
13. C Colome R Artuch MA Vilaseca C Sierra N Brandi N Lambruschini FJ Cambra J Campistol 2003 Lipophilic antioxidants in patients with phenylketonuria Am J Clin Nutr 77 185 188 1:CAS:528:DC%2BD3sXhtFymsQ%3D%3D 12499340 (Pubitemid 36015390)
14. I Dalle-Donne R Rossi D Giustarini A Milzani R Colombo 2003 Protein carbonyl groups as biomarkers of oxidative stress Clin Chim Acta 329 23 38 10.1016/S0009-8981(03)00003-2 1:CAS:528:DC%2BD3sXhtVCktr8%3D 12589963 (Pubitemid 36187411)
15. I Dalle-Donne A Scaloni D Giustarini E Cavarra G Tell G Lungarella R Colombo R Rossi A Milzani 2005 Proteins as biomarkers of oxidative/nitrosative stress in diseases: the contribution of redox proteomics Mass Spectr Rev 24 55 99 10.1002/mas.20006 1:CAS:528:DC%2BD2MXmsFWltg%3D%3D (Pubitemid 40146422)
16. I Dalle-Donne G Aldini M Carini R Colombo R Rossi A Milzani 2006 Protein carbonylation, cellular dysfunction, and disease progression J Cell Mol Med 10 389 406 10.1111/j.1582-4934.2006.tb00407.x 1:CAS:528:DC%2BD2sXlvFCltb0%3D 16796807 (Pubitemid 44870187)
17. MJ Davies S Fu H Wang RT Dean 1999 Stable markers of oxidant damage to proteins and their application in study of human disease Free Radic Biol Med 27 1151 1161 10.1016/S0891-5849(99)00206-3 1:CAS:528:DC%2BD3cXot1Gm 10641706 (Pubitemid 30001474)
18. M Deon A Sitta AG Barschak DM Coelho M Pigatto GO Schmitt LB Jardim R Giugliani M Wajner CR Vargas 2007 Induction of lipid peroxidation and decrease of antioxidant defenses in symptomatic and asymptomatic patients with X-linked adrenoleukodystrophy Int J Dev Neurosci 25 441 447 10.1016/j.ijdevneu.2007.08. 008 1:CAS:528:DC%2BD2sXhtlWktLjO 17928186 (Pubitemid 350086846)
19. N Derin VN Izgut-Uysal A Agac Y Aliciguzel N Demir 2004 l-carnitine protects gastric mucosa by decreasing ischemia reperfusion induced lipid peroxidation J Physiol Pharmacol 55 595 606 1:CAS:528:DC%2BD2cXptlKhtrw%3D 15381830 (Pubitemid 39318851)
20. N Ercal N Aykin-Burns H Gurer-Orhan JD Mcdonald 2002 Oxidative stress in a phenylketonuria animal model Free Radic Biol Med 32 906 911 10.1016/S0891-5849(02)00781-5 1:CAS:528:DC%2BD38XjtVeisr0%3D 11978492 (Pubitemid 34439261)
21. H Esterbauer KH Cheeseman 1990 Determination of aldehydic lipid peroxidation products: malonaldehyde and 4-hydroxynonenal Methods Enzymol 186 407 421 10.1016/0076-6879(90)86134-H 1:CAS:528:DyaK3MXks1Olt7w%3D 2233308 (Pubitemid 20279982)
22. German B (1999) Free radical and antioxidant protocols. Humana Press, Totowa
23. ME Greenberg XM Li BG Gugiu X Gu J Qin RG Salomon SL Hazen 2008 The lipid Whisker model of the structure of oxidized cell membranes J Biol Chem 283 2385 2396 10.1074/jbc.M707348200 1:CAS:528:DC%2BD1cXnsFCktw%3D%3D 18045864
24. MEK Hagen CD Pederzolli AM Sgaravatti R Bridi M Wajner CMD Wanmacher ATS Wyse CS Dutra-Filho 2002 Experimental hyperphenylalaninemia provokes oxidative stress in rat brain Biochim Biophys Acta 1586 344 352 (Pubitemid 34457928)
25. Halliwell B, Gutteridge JMC (2007) 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
26. PR Huttenlocher 2000 The neuropathology of phenylketonuria: human and animal studies Eur J Pediatr 159 102 106 10.1007/PL00014371
27. I Lombeck F Jochum K Terwolbeck 1996 Selenium status in infants and children with phenylketonuria and in maternal phenylketonuria Eur J Pediatr 155 140 144 10.1007/PL00014232
28. OH Lowry NJ Rosebrough A Lewis-Farr RJ Randall 1951 Protein measurement with the Folin phenol reagent J Biol Chem 193 265 275 1:CAS:528: DyaG38XhsVyrsw%3D%3D 14907713
29. WR Markesbery MA Lovell 2007 Damage to lipids, proteins, DNA, and RNA in mild cognitive impairment Arch Neurol 64 954 956 10.1001/archneur.64.7.954 17620484 (Pubitemid 47047994)
30. F Martinez-Cruz D Pozo C Osuna A Espinar C Marchante JM Guerrero 2002 Oxidative stress induced by phenylketonuria in the rat: prevent by melatonin, vitamin E and vitamin C J Neurosci Res 69 550 558 10.1002/jnr.10307 1:CAS:528:DC%2BD38XmtVOgsrg%3D 12210848 (Pubitemid 34831415)
31. PJ Mc Guire A Parikh GA Diaz 2009 Profiling of oxidative stress in patients with inborn errors of metabolism Mol Genet Metab 98 173 180 10.1016/j.ymgme.2009.06.007 1:CAS:528:DC%2BD1MXptlWkur0%3D 19604711
32. DE Paglia WN Valentine 1967 Glutathione peroxidase J Lab Clin Med 70 158 1:CAS:528:DyaF2sXks1Wjur8%3D 6066618
33. PG Pietta 2000 Flavonoids as antioxidants J Nat Prod 63 1035 1042 10.1021/np9904509 1:CAS:528:DC%2BD3cXjsFOrsrY%3D 10924197 (Pubitemid 30624503)
34. P Rajasekar S Kaviarasan CV Anuradha 2005 l-carnitine administration prevents oxidative stress in high fructose-fed insulin resistant rats Diab Croat 34 21 28 (Pubitemid 40942704)
35. AZ Reznick L Packer 1994 Oxidative damage to proteins: spectrophotometric method for carbonyl assay Methods Enzymol 233 357 363 10.1016/S0076-6879(94) 33041-7 1:CAS:528:DyaK2cXmt1Gnt7k%3D 8015470
36. GS Ribas V Manfredini JF de Mari CY Wayhs CS Vanzin GB Biancini A Sitta M Deon M Wajner CR Vargas 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 10.1016/j.ijdevneu.2010.01.002 1:CAS:528:DC%2BC3cXivFGhsb0%3D 20100562
37. KH Schulpis C Nounopoulos A Scarpalezou A Bouloukos S Missiou-Tsagarakis 1990 Serum carnitine level in phenylketonuric children under dietary control in Greece Acta Paediatr Scand 79 930 934 10.1111/j.1651-2227.1990.tb11354.x 1:STN:280:DyaK3M%2FovFyhtA%3D%3D 2264465 (Pubitemid 20333500)
38. KH Schulpis S Tsakiris J Traeger-Synodinos I Papassotiriou 2005 Low total antioxidant status is implicated with high 8-hydroxy-2-deoxyguanosine serum concentrations in phenylketonuria Clin Biochem 38 239 242 10.1016/j.clinbiochem. 2004.11.006 1:CAS:528:DC%2BD2MXhtlSks70%3D 15708545 (Pubitemid 40222323)
39. Scriver CR, Kaufman S (2001) Hyperphenylalaninemia: phenylalanine hydroxylase deficiency. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic and molecular bases of inherited disease. McGraw-Hill, New York, pp 1667-1724
40. C Sierra MA Vilaseca D Moyano N Brandi J Campistol N Lambruschini FJ Cambra R Deulofeu A Mira 1998 Antioxidant status in hyperphenylalaninemia Clin Chim Acta 276 1 9 10.1016/S0009-8981(98)00091-6 1:CAS:528:DyaK1cXltFWktL4%3D 9760015 (Pubitemid 28386824)
41. LR Sirtori CS Dutra-Filho D Fitarelli A Sitta A Haeser AG Barschak M Wajner DM Coelho S Llesuy A Belló-Klein R Giugliani M Deon CR Vargas 2005 Oxidative stress in patients with phenylketonuria Biochim Biophys Acta 1740 68 73 1:CAS:528:DC%2BD2MXjvVKktLo%3D 15878743 (Pubitemid 40692700)
42. A Sitta AG Barschak M Deon T Terroso R Pires R Giugliani CS Dutra-Filho M Wajner CR Vargas 2006 Investigation of oxidative stress parameters in treated phenylketonuric patients Metab Brain Dis 21 287 296 10.1007/s11011-006-9035-0 1:CAS:528:DC%2BD28XhtlWntL3J 17146735 (Pubitemid 46018234)
43. A Sitta AG Barschak M Deon AT Barden GB Biancini PR Vargas CF de Souza C Netto M Wajner CR Vargas 2009 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 10.1016/j.ijdevneu.2009.01.001 1:CAS:528: DC%2BD1MXivVWrsLs%3D 19429389
44. A Sitta AG Barschak M Deon JF De Mari AB Barden C Vanzin GB Biancini IVD Schwartz M Wajner CR Vargas 2009 l-Carnitine blood levels and oxidative stress in treated phenylketonuric patients Cell Mol Nurobiol 29 211 218 10.1007/s10571-008-9313-y 1:CAS:528:DC%2BD1MXhsFWns70%3D
45. DG Smith R Cappai KJ Barnham 2007 The redox chemistry of the Alzheimer's disease amyloid-b peptide Biochim Biophys Acta 1768 1976 1990 10.1016/j.bbamem.2007.02.002 1:CAS:528:DC%2BD2sXosF2gtLw%3D 17433250 (Pubitemid 47125850)
46. K Start 1998 Treating phenylketonuria by a phenylalanine-free diet Prof Care Mother Child 8 109 110 1:STN:280:DyaK1M%2FjtF2juw%3D%3D 9814366
47. MME van Backel G Printzen B Wermuth UN Wiesmann 2000 Antioxidant and thyroid hormone status in selenium-deficient phenylketonuric and hyperphenylalaninemic patients Am J Clin Nutr 72 976 981
48. FJ Van Spronsen PG Smit R Koch 2001 Phenylketonuria: tyrosine beyond the phenylalanine-restricted diet J Inherit Metab Dis 24 1 4 10.1023/A:1005689232358 11286377
49. BC Wilke M Vidailhet A Favier C Guillemin V Ducros J Arnaud MJ Richard 1992 Selenium, glutathione peroxidase (GSH-Px) and lipid peroxidation products before and after selenium supplementation Clin Chim Acta 207 137 142 10.1016/0009-8981(92)90157-L 1:CAS:528:DyaK38XksVOiur8%3D 1591861
50. HA Woo HZ Chae SC Hwang KS Yang SW Kang K Kim SG Rhee 2003 Reversing the inactivation of peroxiredoxin caused by cysteine sulfinic acid formation Science 300 53 656 10.1126/science.1080273 (Pubitemid 36520592)