Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver

International Journal of Developmental Neuroscience

Volumn 27, Issue 4, 2009, Pages 351-356

  Leipnitz, Guilhian ^a   Seminotti, Bianca ^a   Fernandes, Carolina G ^a   Amaral, Alexandre U ^a   Beskow, Ana Paula ^a   da Silva, Lucila de B ^a   Zanatta, Ângela ^a   Ribeiro, César A J ^a   Vargas, Carmen R ^a   Wajner, Moacir ^a,b,c  

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

^b HOSPITAL DE CLÍNICAS DE PORTO ALEGRE   (Brazil)

^c UNIVERSIDADE LUTERANA DO BRASIL   (Brazil)

Author keywords

3 Hydroxy 3 methylglutaric aciduria; Liver; Oxidative stress; Striatum

Indexed keywords

3 HYDROXY 3 METHYLGLUTARIC ACID; 3 HYDROXYISOVALERIC ACID; 3 METHYLGLUTACONATE; 3 METHYLGLUTARATE; GLUTARIC ACID DERIVATIVE; GLUTATHIONE; HYDROXYMETHYLGLUTARYL COENZYME A LYASE; REACTIVE OXYGEN METABOLITE; THIOBARBITURIC ACID REACTIVE SUBSTANCE; UNCLASSIFIED DRUG; VALERIC ACID DERIVATIVE;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BRAIN DAMAGE; BRAIN METABOLISM; CONTROLLED STUDY; CORPUS STRIATUM; ENZYME DEFICIENCY; LIPID PEROXIDATION; LIVER INJURY; LIVER METABOLISM; MALE; NONHUMAN; OXIDATIVE STRESS; PRIORITY JOURNAL; PROTEIN SECRETION; RAT;

ANIMALS; CORPUS STRIATUM; GLUTARATES; GLUTATHIONE; GLYCINE; HUMANS; LIVER; MALE; MEGLUTOL; OXIDATIVE STRESS; OXO-ACID-LYASES; RATS; RATS, WISTAR; THIOBARBITURIC ACID REACTIVE SUBSTANCES; VALERATES;

RATTUS;

EID: 67349164287     PISSN: 07365748     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.ijdevneu.2009.03.001     Document Type: Article

References (60)

1. Aksenov M.Y., and Markesbery W.R. Change in thiol content and expression of glutathione redox system gene in the hippocampus and cerebellum in Alzheimer's disease. Neuroscience Letters 302 (2001) 141-145
2. Artuch R., Colome C., Sierra C., Brandi N., Lambruschini N., Campistol J., Ugarte D., and Vilaseca M.A. A longitudinal study of antioxidant status in phenylketonuric patients. Clinical Biochemistry 37 (2004) 198-203
3. Barschak A.G., Sitta A., Deon M., de Oliveira M.H., Haeser M., Dutra-Filho C.S., Wajner M., and Vargas C.R. Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease. Metabolic Brain Disease 21 (2006) 279-286
4. Beard J.L., Connor J.R., and Jones B.C. Iron in the brain. Nutrition Reviews 51 6 (1993) 157-170
5. Behl C., and Moosmann B. Oxidative nerve cell death in Alzheimer's disease and stroke: antioxidants as neuroprotective compounds. Biological Chemistry 383 (2002) 521-536
6. Berg D., and Youdim M.B. Role of iron in neurodegenerative disorders. Topics in Magnetic Resonance Imaging 17 (2006) 5-17
7. Bogdanov M.B., Andreassen O.A., Dedeoglu A., Ferrante R.J., and Beal M.F. Increased oxidative damage to DNA in a transgenic mouse of Huntington's disease. Journal of Neurochemistry 79 (2001) 1246-1249
8. Bonafe L., Troxler H., Kuster T., Heizmann C.W., Chamoles N.A., Burlina A.B., and Blau N. Evaluation of urinary acylglycines by electrospray tandem mass spectrometry in mitochondrial energy metabolism defects and organic acidurias. Molecular Genetics and Metabolism 69 (2000) 302-311
9. Browne R.W., and Armstrong D. Reduced glutathione and glutathione disulfide. Methods in Molecular Biology 108 (1998) 347-352
10. Castoldi A.F., Coccini T., Ceccatelli S., and Manzo L. Neurotoxicity and molecular effects of methylmercury. Brain Research Bulletin 55 (2001) 197-203
11. Colome C., Artuch R., Vilaseca M.A., Sierra C., Brandi N., Lambruschini N., Cambra F.J., and Campistol J. Lipophilic antioxidants in patients with phenylketonuria. The American Journal of Clinical Nutrition 77 (2003) 185-188
12. de Oliveira Marques F., Hagen M.E., Pederzolli C.D., Sgaravatti A.M., Durigon K., Testa C.G., Wannmacher C.M.D., Wyse A.T.S., Wajner M., and Dutra-Filho C.S. Glutaric acid induces oxidative stress in brain of young rats. Brain Research 964 (2003) 153-158
13. Esterbauer H., and Cheeseman K.H. Determination of aldehydic lipid peroxidation products: malonaldehyde and 4-hydroxynonenal. Methods in Enzymology 186 (1990) 407-421
14. Evelson P., Travacio M., Repetto M., Escobar J., Llesuy S., and Lissi E. Evaluation of total reactive antioxidant potential (TRAP) of tissue homogenates and their cytosols. Archives of Biochemistry and Biophysics 388 2 (2001) 261-266
15. Ferris N.J., and Tien R.D. Cerebral MRI in 3-Hydroxy-3-methylglutaryl coenzyme A lyase deficiency: case report. Neuroradiology 35 (1993) 559-560
16. Fontella F.U., Pulrolnik V., Gassen E., Wannmacher C.M., Klein A.B., Wajner M., and Dutra-Filho C.S. Propionic and l-methylmalonic acids induce oxidative stress in brain of young rats. Neuroreport 11 (2000) 541-544
17. Funghini S., Pasquini E., Cappellini M., Donati M.A., Morrone A., Fonda C., and Zammarchi E. 3-Hydroxy-3-methylglutaric aciduria in an Italian patient is caused by a new nonsense mutation in the HMGDL gene. Molecular Genetics and Metabolism 73 (2001) 268-275
18. Gibson K.M., Breuer J., and Nyhan W.L. 3-Hydroxy-3-methylglutarylcoenzyme A lyase deficiency: review of 18 reported patients. European Journal of Pediatrics 148 (1988) 180-186
19. Gibson K.M., Cassidy S.B., Seaver L.H., Wanders R.J., Kennaway N.G., Mitchell G.A., and Spark R.P. Fatal cardiomyopathy associated with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency. Journal of Inherited Metabolic Disease 17 (1994) 291-304
20. Gordon K., Riding M., Camfild P., Bawden H., Ludman M., and Bagnell P. CT and MR of 3-Hydroxy-3-methylglutaryl coenzyme A lyase deficiency. American Journal of Neuroradiology 15 (1994) 1474-1476
21. Hallbergson A.F., Gnatenco C., and Peterson D.A. Neurogenesis and brain injury: managing a renewable resource for repair. The Journal of Clinical Investigation 112 (2003) 1128-1133
22. Halliwell B., and Gutteridge J.M.C. Measurement of reactive species. In: Halliwell B., and Gutteridge J.M.C. (Eds). Free Radicals in Biology and Medicine. 4th ed. (2007), Oxford University Press, Oxford 268-340
23. Halliwell B., and Gutteridge J.M.C. Cellular responses to oxidative stress: adaptation, damage, repair, senescence and death. In: Halliwell B., and Gutteridge J.M.C. (Eds). Free Radicals in Biology and Medicine. 4th ed. (2007), Oxford University Press, Oxford 268-340
24. Jafari M. Dose- and time-dependent effects pf sulfur mustard on antioxidant system in liver and brain of rat. Toxicology 231 (2007) 30-39
25. Kaushik S., and Kaur J. Chronic cold exposure affects the antioxidant defense system in various rat tissues. Clinica Chimica Acta 333 (2003) 69-77
26. Kienzle Hagen M.E., Pederzolli C.D., Sgaravatti A.M., Bridi R., Wajner M., Wannmacher C.M.D., Wyse A.T., and Dutra-Filho C.S. Experimental hyperphenylalaninemia provokes oxidative stress in rat brain. Biochimica et Biophysica Acta 1586 (2002) 344-352
27. Kolker S., Ahlemeyer B., Krieglstein J., and Hoffmann G.F. Contribution of reactive oxygen species to 3-hydroxyglutarate neurotoxicity in primary neuronal cultures from chick embryo telencephalons. Pediatric Research 50 (2001) 76-82
28. Latini A., Scussiato K., Rosa R.B., Leipnitz G., Llesuy S., Belló-Klein A., Dutra-Filho C.S., and Wajner M. Induction of oxidative stress by L-2-hydroxyglutaric acid in rat brain. Journal of Neuroscience Research 74 1 (2003) 103-110
29. Latini A., Scussiato K., Rosa R.B., Llesuy S., Belló-Klein A., Dutra-Filho C.S., and Wajner M. D-2-hydroxyglutaric acid induces oxidative stress in cerebral cortex of young rats. European Journal of Neuroscience 17 10 (2003) 2017-2022
30. Latini A., Scussiato K., Leipnitz G., Dutra-Filho C.S., and Wajner M. Promotion of oxidative stress by 3-hydroxyglutaric acid in rat striatum. Journal of Inherited Metabolic Disease 28 (2005) 57-67
31. Latini A., Ferreira G.C., Scussiato K., Schuck P.F., Solano A.F., Dutra-Filho C.S., Vargas C.R., and Wajner M. Induction of oxidative stress by chronic and acute glutaric acid administration to rats. Cellular and Molecular Neurobiology 27 4 (2007) 423-438
32. Lee C., Tsai F.J., Wu J.Y., Peng C.T., Tsai C.H., Hwu W.L., Wang T.R., and Millington D.S. 3-Hydroxy-3-methylglutaric aciduria presenting with Reye like syndrome: report of one case. Acta Paediatrica Taiwan 40 (1999) 445-447
33. Leipnitz G., Seminotti B., Haubrich J., Dalcin M.B., Dalcin K.B., Solano A., de Bortoli G., Rosa R.B., Amaral A.U., Dutra-Filho C.S., Latini A., and Wajner M. 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 86 (2008) 683-693
34. Leipnitz G., Seminotti B., Amaral A.U., de Bortoli G., Solano A., Schuck P.F., Wyse A.T.S., Wannmacher C.M.D., Latini A., and Wajner M. Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats. Life Sciences 82 (2008) 652-662
35. Lowry O.H., Rosebrough N.J., Farr A.L., and Randall R.J. Protein measurement with the Folin phenol reagent. Journal of Biological Chemistry 193 (1951) 265-275
36. Mancuso M., Coppede F., Migliore L., Siciliano G., and Murri L. Mitochondrial dysfunction, oxidative stress and neurodegeneration. Journal of Alzheimer's Disease 10 (2006) 59-73
37. Mori N., Yasutake A., and Hirayama K. Comparative study of activities in reactive oxygen species production/defense system in mitochondria of rat brain and liver, and their susceptibility to methylmercury toxicity. Archives of Toxicology 81 (2007) 769-776
38. Pettenuzzo L.F., Schuck P.F., Fontella F., Wannmacher C.M., Wyse A.T., Dutra-Filho C.S., Netto C.A., and Wajner M. Ascorbic acid prevents cognitive deficits caused by chronic administration of propionic acid to rats in the water maze. Pharmacology, Biochemistry and Behavior 73 (2002) 623-629
39. Pettenuzzo L.F., Schuck P.F., Wyse A.T., Wannmacher C.M., Dutra-Filho C.S., Netto C.A., and Wajner M. Ascorbic acid prevents water maze behavioral deficits caused by early postnatal methylmalonic acid administration in the rat. Brain Research 976 (2003) 234-242
40. Pospisilova E., Mrazova L., Hrda J., Martincova O., and Zeman J. Biochemical and molecular analyzes in three patients with 3-hydroxy-3-methylglutaric aciduria. Journal of Inherited Metabolic Disease 26 (2003) 433-441
41. Pushpendran C.K., Subramanian M., and Devasagayam T.P. Developmental changes in the peroxidation potential of rat brain homogenate and mitochondria. Mechanisms of Ageing and Development 73 (1994) 197-208
42. Rigo F.K., Pasquetti L., Malfatti C.R., Fighera M.R., Coelho R.C., Petri C.Z., and Mello C.F. Propionic acid induces convulsions and protein carbonylation in rats. Neuroscience Letters 408 (2006) 151-154
43. Ristoff E., and Larsson A. Oxidative stress in inborn errors of metabolism: lessons from glutathione deficiency. Journal of Inherited Metabolic Disease 25 (2002) 223-226
44. Sarafian T.A., Bredesen D.E., and Verity M.A. Cellular resistance to methylmercury. Neurotoxicology 17 (1996) 23-36
45. Sgaravatti A.M., Sgarbi M.B., Testa C.G., Durigon K., Pederzolli C.D., Prestes C.C., Wyse A.T.S., Wannmacher C.M.D., Wajner M., and Dutra-Filho C.S. γ-Hydroxybutyric acid induces oxidative stress in cerebral cortex of young rats. Neurochemistry International 50 (2007) 564-570
46. Sierra C., Vilaseca M.A., Moyano D., Brandi N., Campistol J., Lambruschini N., Cambra F.J., Deulofeu R., and Mira A. Antioxidant status in hyperphenylalaninemia. Clinica Chimica Acta 276 (1998) 1-9
47. Singh P., Jain A., and Kaur G. Impact of hypoglycemia and diabetes on CNS: Correlation of mitochondrial oxidative stress with DNA damage. Molecular and Cellular Biochemistry 260 (2004) 153-159
48. Sirtori L.R., Dutra-Filho C.S., Fitarelli D., Sitta A., Haeser A., Barschak A.G., Wajner M., Coelho D.M., Llesuy S., Bello-Klein A., Giugliani R., Deon M., and Vargas C.R. Oxidative stress in patients with phenylketonuria. Biochimica et Biophysica Acta 1740 (2005) 68-73
49. Sitta A., Barschak A.G., Deon M., Terroso T., Pires R., Giugliani R., Dutra-Filho C.S., Wajner M., and Vargas C.R. Investigation of oxidative stress parameters in treated phenylketonuric patients. Metabolic Brain Disease 21 (2006) 287-296
50. Stoy N., Mackay G.M., Forrest C.M., Christofides J., Egerton M., Stone T.W., and Darlington L.G. Tryptophan metabolism and oxidative stress in patients with Huntington's disease. Journal of Neurochemistry 93 (2005) 611-623
51. Suh S.W., Gum E.T., Hamby A.M., Chan P.H., and Swanson R.A. Hypoglycemic neuronal death is triggered by glucose reperfusion and activation of neuronal NADPH oxidase. The Journal of Clinical Investigation 117 4 (2007) 910-918
52. Sweetman L., and Williams J.C. Branched chain organic acidurias. In: Scriver C.R., Beaudet A.L., Sly W.S., and Valle D. (Eds). The Metabolic and Molecular Bases of Inherited Disease (2001), McGraw-Hill, New York 2340-2342
53. Tarla M.R., Ramalho F., Ramalho L.N., Silva T.C., Brandão D.F., Ferreira J., Silva O.C., and Zucoloto S. Cellular aspects of liver regeneration. Acta Cirurgica Brasileira 21 (2006) 63-66
54. van der Knaap M.S., Bakker H.D., and Valk J. MR imaging and proton spectroscopy in 3-hydroxy-3-methylglutaryl coenzyme A lyase deficiency. American Journal of Neuroradiology 19 (1998) 378-382
55. Vargas C.R., Sitta A., Schmitt G., Ferreira G.C., Cardoso M.L., Coelho D., Gibson K.M., and Wajner M. Incidence of 3-hydroxy-3-methylglutaryl-coenzyme A lyase (HL) deficiency in Brazil, South America. Journal of Inherited Metabolic Disease 31 1 (2008) 127-128
56. Wysocki S.J., and Hahnel R. 3-Hydroxy-3-methylglutaryl-coenzyme a lyase deficiency: a review. Journal of Inherited Metabolic Disease 9 (1986) 225-233
57. Yalcinkaya C., Dincer A., Gündüz E., Ficicioglu C., Kocer N., and Aydin A. MRI and MRS in HMG-CoA lyase deficiency. Pediatric Neurology 20 (1999) 375-380
58. Yylmaz Y., Ozdemir N., Ekinci G., Baykal T., and Kocaman C. Corticospinal tract involvement in a patient with 3-HMG coenzyme A lyase deficiency. Pediatric Neurology 35 (2006) 139-141
59. Zafeiriou D.I., Vargiami E., Mayapetek E., Augoustidou-Savvopoulou P., and Mitchell G.A. 3-Hydroxy-methylglutaryl Coenzyme A lyase deficiency with reversible white matter changes after treatment. Pediatric Neurology 37 1 (2007) 47-50
60. Zaleska M.M., and Floyd R.A. Regional lipid peroxidation in rat brain in vitro: possible role of endogenous iron. Neurochemical Research 10 (1985) 397-410