Protein and lipid damage in maple syrup urine disease patients: L-carnitine effect

International Journal of Developmental Neuroscience

Volumn 31, Issue 1, 2013, Pages 21-24

  Mescka, Caroline Paula ^b   Wayhs, Carlos Alberto Yasin ^b   Vanzin, Camila Simioni ^b   Biancini, Giovana Brondani ^b   Guerreiro, Gilian ^a   Manfredini, Vanusa ^c   Souza, Carolina ^a   Wajner, Moacir ^a,b   Dutra filho, Carlos Severo ^b   Vargas, Carmen Regla ^a,b  

^a HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

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

^c UNIVERSIDADE FEDERAL DO PAMPA   (Brazil)

Author keywords

Antioxidant; L Carnitine; Lipid peroxidation; Maple syrup urine disease

Indexed keywords

BRANCHED CHAIN AMINO ACID; CARBONYL DERIVATIVE; CARNITINE; LEUCINE; MALONALDEHYDE;

ANTIOXIDANT ACTIVITY; ARTICLE; BLOOD SAMPLING; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; DIET RESTRICTION; DIET SUPPLEMENTATION; DOWN REGULATION; ENERGY METABOLISM; HUMAN; LIPID PEROXIDATION; MAPLE SYRUP URINE DISEASE; OXIDATIVE STRESS; PRIORITY JOURNAL; PROTEIN DEFECT; PROTEIN DIET; SCHOOL CHILD; TREATMENT OUTCOME;

AMINO ACIDS; ANALYSIS OF VARIANCE; CARNITINE; CHILD; CHILD, PRESCHOOL; FEMALE; HUMANS; LIPID METABOLISM; MALE; MALONDIALDEHYDE; MAPLE SYRUP URINE DISEASE; PROTEIN CARBONYLATION; PROTEINS; VITAMIN B COMPLEX;

ACER;

EID: 84870525015     PISSN: 07365748     EISSN: 1873474X     Source Type: Journal    
DOI: 10.1016/j.ijdevneu.2012.10.109     Document Type: Article

References (23)

1. Barschak A.G., Sitta A., Deon M., Oliveira M.H., Haeser A., Dutra-Filho C.S., Wajner M., Vargas C.R. Evidence that oxidative stress is increased in plasma from patients with Maple Syrup Urine Disease. Metabolic Brain Disease 2006, 21:279-286.
2. Barschak A.G., Sitta A., Deon M., Barden A.T., Dutra-Filho C.S., Wajner M., Vargas C.R. Oxidative stress in plasma from maple syrup urine disease patients during treatment. Metabolic Brain Disease 2008, 23:71-80.
3. Bridi R., Araldi J., Sgarbi M.B., Testa C.G., Durigon K., Wajner M., Dutra-Filho C.S. Induction of oxidative stress in rat brain by the metabolites accumulating in maple syrup urine disease. International Journal of Developmental Neuroscience 2003, 21:327-332.
4. Chace D.H., Hillman S.L., Van Hove J.L., Naylor E.W. Rapid diagnosis of MCAD deficiency: quantitative analysis of octanoylcarnitine and other acylcarnitines in newborn blood spots by tandem mass spectrometry. Clinical Chemistry 1997, 43:2106-2113.
5. Chuang D.T., Shih V.E. Maple syrup urine disease (branched-chain ketoaciduria). The Metabolic and Molecular Bases of Inherited Disease 2001, 1971-2005. McGraw-Hill, New York. C.R. Scriver, A.L. Beaudt, W.L. Sly, D. Valle (Eds.).
6. Dalle-Donne I., Aldini G., Carini M., Colombo R., Rossi R., Milzani A. Protein carbonylation, cellular dysfunction, and disease progression. Journal of Cellular and Molecular Medicine 2006, 10:389-406.
7. Derin N., Izgut-Uysal V.N., Agac A., Aliciguzel Y., Demir N. l-Carnitine protects gastric mucosa by decreasing ischemia - reperfusion induced lipid peroxidation. Journal of Physiology and Pharmacology 2004, 55:595-606.
8. Fontella F.U., Gassen E., Pulrolnik V., Wannmacher C.M.D., Klein A.B., Wajner M., Dutra C.S. Stimulation of lipid peroxidation in vitro in rat brain by metabolites accumulating in maple syrup urine disease. Metabolic Brain Disease 2002, 17:47-54.
9. Gulcin I. Antioxidant and antiradical activities of l-carnitine. Life Sciences 2006, 78:803-811.
10. Halliwell B., Gutteridge M.C. Free Radicals in Biology and Medicine 2007, Oxford University Press Inc., New York. 4th ed.
11. Joseph M.H., Marsden C.A. Amino acids and small peptides. HPLC of Small Peptides 1986, 13-27. IRL Press, Oxford. C.F. Lim (Ed.).
12. Karatepe M. Simultaneous determination of ascorbic acid and free malondialdehyde in human serum by HPLC-UV. LCGC North America 2004, 22:362-365.
13. Levine R.L., Garland D., Oliver C.N., Amici A., Climent L., Lenz A.G., Ahn B.W., Shalthiel S., Stadman E.R. Determination of carbonyl content in oxidatively modified proteins. Methods in Enzymology 1990, 186:464-478.
14. Mescka C., Moraes T., Rosa A., Mazzola P., Piccoli B., Jacques C., Dalazen G., Coelho J., Cortes M., Terra M., Regla Vargas C., Dutra-Filho C.S. In vivo neuroprotective effect of l-carnitine against oxidative stress in maple syrup urine disease. Metabolic Brain Disease 2011, 26:21-28.
15. Muthuswamy A.D., Vedagiri K., Ganesan M., Chinnakannu P. Oxidative stress-mediated macromolecular damage and dwindle in antioxidant status in aged rat brain regions: role of l-carnitine and dl-alpha-lipoic acid. Clinica Chimica Acta 2006, 368:84-92.
16. Rani P.J., Panneerselvam C. Effect of l-carnitine on brain lipid peroxidation and antioxidant enzymes in old rats. Journals of Gerontology Series A: Biological Sciences and Medical Sciences 2003, 57:134-137.
17. Requena J.R., Levine R.L., Stadtman E.R. Recent advances in the analysis of oxidized proteins. Amino Acids 2003, 25:221-226.
18. Ribas G.S., Manfredini V., De Mari J.F., Wayhs C.Y., Vanzin C.S., Biancini G.B., Sitta A., Deon M., Wajner M., Vargas C.R. Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of l-carnitine supplementation. International Journal of Developmental Neuroscience 2010, 28:127-132.
19. Sitta A., Vanzin C.S., Biancini G.B., Manfredini V., de Oliveira A.B., Wayhs C.A., Ribas G.O., Giugliani L., Schwartz I.V., Bohrer D., Garcia S.C., Wajner M., Vargas C.R. Evidence that L-carnitine and selenium supplementation reduces oxidative stress in phenylketonuric patients. Cellular and Molecular Neurobiology 2011, 31:429-436.
20. Sitta A., Barschak A.G., Deon M., Mari J., Barden A.T., Vanzin C.S., Biancini G.B., Schwartz I.V., Wajner M., Vargas C.R. l-Carnitine blood levels and oxidative stress in treated phenylketonuric patients. Cellular and Molecular Neurobiology 2009, 29:211-218.
21. Stadtman E.R., Levine R.L. Free-radical mediated oxidation of free amino acids and amino acid residues in proteins. Amino Acids 2003, 25:207-218.
22. Tastekin N., Aydogdu N., Dokmeci D., Usta U., Birtane M., Erbas H., Ture M. Protective effects of l-carnitine and alpha-lipoic acid in rats with adjuvant arthritis. Pharmacological Research 2007, 56:303-310.
23. Wajner M., Coelho D.M., Barschak A.G., Araujo P.R., Pires R.F., Lulhier F.L., Vargas C.R. Reduction of large neutral amino acid concentrations in plasma and CSF of patients with maple syrup urine disease during crises. Journal of Inherited Metabolic Disease 2000, 23:505-512.