Evidence that 3-hydroxy-3-methylglutaric acid promotes lipid and protein oxidative damage and reduces the nonenzymatic antioxidant defenses in rat cerebral cortex

Journal of Neuroscience Research

Volumn 86, Issue 3, 2008, Pages 683-693

  Leipnitz, Guilhian ^a   Seminotti, Bianca ^a   Haubrich, Josué ^a   Dalcin, Manuela B ^a   Dalcin, Karina B ^a   Solano, Alexandre ^a   De Bortoli, Giorgia ^a   Rosa, Rafael B ^a   Amaral, Alexandre U ^a   Dutra Filho, Carlos S ^a   Latini, Alexandra ^b   Wajner, Moacir ^a,b,c,d  

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

^b FEDERAL UNIVERSITY OF SANTA CATARINA   (Brazil)

^c HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

^d UNIVERSIDADE LUTERANA DO BRASIL   (Brazil)

Author keywords

3 hydroxy 3 methylglutaric acid; 3 hydroxy 3 methylglutaric aciduria; Oxidative stress

Indexed keywords

3 HYDROXY 3 METHYLGLUTARIC ACID; ACETYLCYSTEINE; ALPHA TOCOPHEROL; CATALASE; GLUTATHIONE; MELATONIN; SUPEROXIDE DISMUTASE; THIOBARBITURIC ACID;

ANTIOXIDANT ACTIVITY; ARTICLE; BRAIN CORTEX; CHEMOLUMINESCENCE; CONTROLLED STUDY; IN VITRO STUDY; LIPID PEROXIDATION; NONHUMAN; OXIDATIVE STRESS; PRIORITY JOURNAL; RAT;

ANIMALS; ANTIOXIDANTS; CEREBRAL CORTEX; DOWN-REGULATION; GLUTATHIONE; LIPID PEROXIDATION; MEGLUTOL; NERVE TISSUE PROTEINS; OXIDATION-REDUCTION; OXIDATIVE STRESS; OXIDOREDUCTASES; RATS; RATS, WISTAR; SUPEROXIDES;

EID: 38849174972     PISSN: 03604012     EISSN: 10974547     Source Type: Journal    
DOI: 10.1002/jnr.21527     Document Type: Article

References (53)

1. Aebi H. 1984. Catalase, in vitro. Methods Enzymol 105:121-126.
2. Aksenov MY, Markesbery WR. 2001. Change in thiol content and expression of glutathione redox system gene in the hippocampus and cerebellum in Alzheimer's disease. Neurosci Lett 302:141-145.
3. Anisimov VN, Popovich IG, Zabezhinski MA, Anisimov SV, Vesnushkin GM, Vinogradova IA. 2006. Melatonin as antioxidant, geroprotector and anticarcinogen. Biochim Biophys Acta 1757:573-589.
4. Behl C, Moosmann B. 2002. Oxidative nerve cell death in Alzheimer's disease and stroke: antioxidants as neuroprotective compounds. Biol Chem 383:521-536.
5. Berg D, Youdim MB. 2006. Role of iron in neurodegenerative disorders. Top Magn Reson Imaging 17:5-17.
6. Bogdanov MB, Andreassen OA, Dedeoglu A, Ferrante RJ, Beal MF. 2001. Increased oxidative damage to DNA in a transgenic mouse of Huntington's disease. J Neurochem 79:1246-1249.
7. Bonafë L, Troxler H, Kuster T, Heizmann CW, Chamoles NA, Burlina AB, Blau N. 2000. Evaluation of urinary acylglycines by electrospray tandem mass spectrometry in mitochondrial energy metabolism defects and organic acidurias. Mol Genet Metab 69:302-311.
8. Browne RW, Armstrong D. 1998. Reduced glutathione and glutathione disulfide. Methods Mol Biol 108:347-352.
9. Esterbauer H, Cheeseman KH. 1990. Determination of aldehydic lipid peroxidation products: malonaldehyde and 4-hydroxynonenal. Methods Enzymol 186:407-421.
10. Evelson P, Travacio M, Repetto M, Escobar J, Llesuy S, Lissi E. 2001. Evaluation of total reactive antioxidant potential (TRAP) of tissue homogenates and their cytosols. Arch Biochem Biophys 388:261-266.
11. Faull KF, Bolton PD, Halpern B, Hammond J, Dankes DM. 1976. The urinary organic acid profile associated with 3-hydroxy-3-methylglutaric aciduria. Clin Chim Acta 73:553-559.
12. Funghini S, Pasquini E, Cappellini M, Donati MA, Morrone A, Fonda C, Zammarchi E. 2001. 3-Hydroxy-3-methylglutaric aciduria in an kalian patient is caused by a new nonsense mutation in the HMGDL gene. Mol Genet Metab 73:268-275.
13. Genet S, Kale RK, Baquer NZ. 2002. Alterations in antioxidant enzymes and oxidative damage in experimental diabetic rat tissues: Effect of vanadate and fenugreek (Trigonella foenum graecum). Mol Cell Biochem 236:7-12.
14. Gibson KM, Breuer J, Kaiser K, Nyhan WL, McCoy EE, Ferreira P, Greene CL, Blitzer MG, Shapira E, Reverte F, Conde C, Bagnell P, Cole DEC. 1988a. 3-Hydroxy-3-methylglutarylcoenzyme A lyase deficiency: report of five new patients. J Inherit Metab Dis 11:76-87.
15. Gibson KM, Breuer J, Nyhan WL. 1988b. 3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: review of 18 reported patients. Eur J Pediatr 148:180-186.
16. Gibson KM, Cassidy SB, Seaver LH, Wanders RJ, Kennaway NG, Mitchell GA, Spark RP. 1994. Fatal cardiomyopathy associated with 3-hydroxy-3-methylglutaryl- CoA lyase deficiency. J Inherit Metab Dis 17:291-304.
17. Gonzalez-Flecha B, Llesuy S, Boveris A. 1991. Hydroperoxide-initiated chemiluminescence: an assay for oxidative stress in biopsies of heart, liver, and muscle. Free Radic Biol Med 10:93-100.
18. Halliwell B, Gutteridge JMC. 1996. Oxygen radicals and nervous system. Trends Neurosci 8:22-26.
19. Halliwell B, Gutteridge JMC. 2007a. Measurement of reactive species. In: Halliwell B, Gutteridge JMC, editors. Free radicals in biology and medicine. Oxford: Oxford University Press. p 268-340.
20. Halliwell B, Gutteridge JMC. 2007b. Cellular responses to oxidative stress: adaptation, damage, repair, senescence and death. In: Halliwell B, Gutteridge JMC, editors. Free radicals in biology and medicine. Oxford: Oxford University Press. p 187-267.
21. Hoffmann GF, Meier-Augenstein W, Stockier S, Surtees R, Rating D, Nyhan WL. 1993. Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis 16:648-669.
22. Hoffmann GF, Gibson KM, Trefz FK, Nyhan WL, Bremer HJ, Rating D. 1994. Neurological manifestations of organic acid disorders. Eur J Pediatr 153:S94-S100.
23. Jafari M. 2007. Dose- and time-dependent effects of sulfur mustard on antioxidant system in liver and brain of rat. Toxicology 231:30-39.
24. Karelson E, Bogdanovic N, Garlind A, Winblad B, Zilmer K, Kullisaar T, Vihalemm T, Kairane C, Zilmer M. 2001. The cerebrocortical areas in normal brain aging and in the Alzheimer's disease: noticeable difference in the lipid peroxidation level and in antioxidant defense. Neurochem Res 26:353-361.
25. Kowakowski AJ, Vercesi AE, Castilho RF. 1997. Mitochondrial membrane protein thiol reactivity with N-ethylmaleimide or mersalyl is modified by Ca2+: correlation with mitochondrial permeability transition. Biochim Biophys Acta 1318:395-402.
26. Lee C, Tsai FJ, Wu JY, Peng CT, Tsai CH, Hwu WL, Wang TR, Millington DS. 1999. 3-Hydroxy-3-methylglutaric aciduria presenting with Reye like syndrome: report of one case. Acta Paediatr Taiwan 40:445-447.
27. Levine RL. 2002. Carbonyl modified proteins in cellular regulation, aging, and disease. Free Radic Biol Med 32:790-796.
28. Lissi E, Pascual C, Del Castillo MD. 1992. Luminol luminescence induced by 2,2-azo-bis-(2-amidinopropane) thermolysis. Free Radic Res Commun 17:299-311.
29. Lissi E, Sahm-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.
30. Lowry OH, Rosebrough NJ, Lewis-Farr A, Randall RJ. 1951. Protein measurement with the Folin phenol reagent. J Biol Chem 193:265-275.
31. Lyon G, Adams RD, Kolodny EH. 1996. The neurology of neonatal hereditary metabolic diseases. In: Lyon G, Adams RD, Kolodny EH, editors. Neurology of hereditary metabolic disease of children. New York: McGraw Hill. p 29-30.
32. Mancuso M, Coppede F, Migliore L, Siciliano G, Murri L. 2006. Mitochondrial dysfunction, oxidative stress and neurodegeneration. J Alzheimer Dis 10:59-73.
33. Marklund SL. 1985. Pyrogallol autoxidation. In: Handbook for oxygen radical researh. Boca Raton, FL: CRC Press. p 243-247.
34. Méndez-Álvarez E, Soto-Otero R, Hermida-Aeijeiras A, López-Real AM, Labandeira-García JL. 2001. Effects of aluminium and zinc on the oxidative stress caused by 6-hydroxydopamine autoxidation: relevance for the pathogenesis of Parkinson's disease. Biochim Biophys Acta 1586:155-168.
35. Perez-Severiano F, Rios C, Segovia J. 2000. Striatal oxidative damage parallels the expression of a neurological phenotype in mice transgenic for the mutation of Huntington's disease. Brain Res 862:234-237.
36. Poderoso JJ, Carreras MC, Lisdero C, Riobó N, Schöpfer F, Boveris A. 1996. Nitric oxide inhibits electron transfer and increases superoxide radical production in rat heart mitochondria and submitochondrial particles. Arch Biochem Biophys 328:85-92.
37. Reiter RJ. 1998. Oxidative damage in the central nervous system: protection by melatonin. Prog Neurobiol 56:359-384.
38. Reiter RJ, Carneiro RC, Oh CS. 1997a. Melatonin in relation to cellular antioxidative defense mechanisms. Horm Metab Res 29:363-372.
39. Reiter R, Tang L, Garcia JJ, Munoz-Hoyos A. 1997b. Pharmacological actions of melatonin in oxygen radical pathophysiology. Life Sci 60:2255-2271.
40. Reiter RJ, Tan DX, Manchester LC, Qi W. 2001. Biochemical reactivity of melatonin with reactive oxygen and nitrogen species: a review of the evidence. Cell Biochem Biophys 34:237-256.
41. Reznick AZ, Packer L. 1993. Free radicals and antioxidants in muscular neurological diseases and disorders. In: Poli G, Albano E, Dianzani UM, editors. Free radicals: from basic science to medicine. Basel: Birkhauser Verlag. p 425-437.
42. Reznick AZ, Packer L. 1994. Oxidative damage to proteins: Spectrophotometric method for carbonyl assay. Methods Enzymol 233:357-363.
43. Sauer SW, Okun JG, Flicker G, Mahringer A, Müller I, Crnic LS, Mühlhausen C, Hoffmann GF, Hörster F, Goodman SI, Harding CO, Koeller DM, Kölker S. 2006. Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency. J Neurochem 97:899-910.
44. Stellmer F, Keyser B, Burckhardt BC, Koepsell H, Streichen T, Glatzel M, Jabs S, Thiem J, Herdering W, Koeller DM, Goodman SI, Lukacs Z, Ullrich K, Burckhardt G, Braulke T, Muhlhausen C. 2007. 3-Hydroxyglutaric acid is transported via the sodium-dependent dicarboxylate transporter NaDC3. J Mol Med (in press).
45. Stoy N, Mackay GM, Forrest CM, Christofides J, Egerton M, Stone TW, Darlington LG. 2005. Tryptophan metabolism and oxidative stress in patients with Huntington's disease. J Neurochem 93:611-623.
46. Sweetman L, Williams JC. 2001. Branched chain organic acidurias. In: Scnver CR, Beaudet AL, Sly WS, Valle D, editors. The metabolic and molecular bases of inherited disease. New York: McGraw-Hill. p 2340-2342.
47. Thompson GN, Chalmers RA, Halliday D. 1990. The contribution of protein catabolism to metabolic decompensation in 3-hydroxy-3-methylglutaric aciduria. Eur J Pediatr 149:346-350.
48. van der Knaap MS, Bakker HD, Valk J. 1998. MR imaging and proton spectroscopy in 3-hydroxy-3-methylglutaryl coenzyme A lyase deficiency. Am J Neuroradiol 19:378-382.
49. Wendel A. 1981. Glutathione peroxidase. Methods Enzymol 177:325-332.
50. Wysocki SJ, Hähnel R. 1986. 3-Hydroxy-3-methylglutaryl-coenzyme a lyase deficiency: a review. J Inherit Metab Dis 9:225-233.
51. Wysocki SJ, Wilkonson SP, Haehnel R, Wong CYB, Panegyres PK. 1976. 3-Hydroxy-3-methylglutaric aciduria, combined with 3-methylglutaconic aciduria. Clin Chim Acta 70:399-406.
52. Yalcinkaya C, Dincer A, Gündüz E, Ficicioglu C, Kocer N, Aydin A. 1999. MRI and MRS in HMG-CoA lyase deficiency. Pediatr Neurol 20:375-380.
53. Yylmaz Y, Ozdemir N, Ekinci G, Baykal T, Kocaman C. 2006. Corticospinal tract involvement in a patien with 3-HMG coenzyme A lyase deficiency. Pediatr Neurol 35:139-141.