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Volumn 47, Issue 12, 2013, Pages 1066-1075

Redox homeostasis is compromised in vivo by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in rat cerebral cortex and liver

Author keywords

3 hydroxy 3 methylglutaric acid; 3 methylglutaric acid; Hepatopathy; Neurological symptoms; Oxidative stress

Indexed keywords

3 HYDROXY 3 METHYLGLUTARIC ACID; 3 METHYLGLUTARIC ACID; CARBONYL DERIVATIVE; CATALASE; DICHLOROFLUORESCEIN; GLUTATHIONE; GLUTATHIONE PEROXIDASE; HYDROXYMETHYLGLUTARYL COENZYME A LYASE; LYSINE; NITRATE; NITRITE; SUPEROXIDE DISMUTASE;

EID: 84887482387     PISSN: 10715762     EISSN: 10292470     Source Type: Journal    
DOI: 10.3109/10715762.2013.853876     Document Type: Article
Times cited : (20)

References (47)
  • 1
    • 0003237157 scopus 로고    scopus 로고
    • Branched chain organic acidurias
    • Scriver CR, Beaudet AL, Sly WS, Valle D (eds.) New York: McGraw-Hill
    • Sweetman L, Williams JC. Branched chain organic acidurias. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds.). The Metabolic and Molecular Bases of Inherited Disease, 8th ed. New York: McGraw-Hill, 2001. pp. 2340-2342.
    • (2001) The Metabolic and Molecular Bases of Inherited Disease, 8th Ed , pp. 2340-2342
    • Sweetman, L.1    Williams, J.C.2
  • 2
    • 0034039710 scopus 로고    scopus 로고
    • Evaluation of urinary acylglycines by electrospray tandem mass spectrometry in mitochondrial energy metabolism defects and organic acidurias
    • DOI 10.1006/mgme.2000.2982
    • Bonafé L, Troxler H, Kuster T, Heizmann CW, Chamoles NA, Burlina AB, Blau N. Evaluation of urinary acylglycines by electrospray tandem mass spectrometry in mitochondrial energy metabolism defects and organic acidurias. Mol Genet Metab 2000; 69: 302-311. (Pubitemid 30333665)
    • (2000) Molecular Genetics and Metabolism , vol.69 , Issue.4 , pp. 302-311
    • Bonafe, L.1    Troxler, H.2    Kuster, T.3    Heizmann, C.W.4    Chamoles, N.A.5    Burlina, A.B.6    Blau, N.7
  • 3
    • 0025099536 scopus 로고
    • The contribution of protein catabolism to metabolic decompensation in 3-hydroxy-3-methylglutaric aciduria
    • Thompson GN, Chalmers RA, Halliday D. The contribution of protein catabolism to metabolic decompensation in 3-hydroxy-3-methylglutaric aciduria. Eur J Pediatr 1990; 149: 346-350. (Pubitemid 20058569)
    • (1990) European Journal of Pediatrics , vol.149 , Issue.5 , pp. 346-350
    • Thompson, G.N.1    Chalmers, R.A.2    Halliday, D.3
  • 4
    • 0018316346 scopus 로고
    • Lethal hypoglycemia in a child with a deficiency of 3-hydroxy-3- methylglutarylcoenzyme A lyase
    • Schutgens RB, Heymans H, Ketel A, Veder HA, Duran M, Ketting D, Wadman SK. Lethal hypoglycemia in a child with a deficiency of 3-hydroxy-3- methylglutarylcoenzyme A lyase. J Pediatr 1979; 94: 89-91. (Pubitemid 9036142)
    • (1979) Journal of Pediatrics , vol.94 , Issue.1 , pp. 89-91
    • Schutgens, R.B.H.1    Heymans, H.2    Ketel, A.3
  • 5
    • 0022995791 scopus 로고
    • 3-Hydroxy-3-methylglutarylcoenzyme a lyase deficiency: A review
    • Wysocki SJ, Hähnel R. 3-Hydroxy-3-methylglutarylcoenzyme a lyase deficiency: a review. J Inherit Metab Dis 1986; 9: 225-233.
    • (1986) J Inherit Metab Dis , vol.9 , pp. 225-233
    • Wysocki, S.J.1    Hähnel, R.2
  • 6
    • 0021842702 scopus 로고
    • Dizygotic twins with 3-hydroxy-3-methylglutaric aciduria; unusual presentation, family studies and dietary management
    • DOI 10.1007/BF00451909
    • Stacey TE, de Sousa C, Tracey BM, Whitelaw A, Mistry J, Timbrell P, Chalmers RA. Dizygotic twins with 3-hydroxy-3-methylglutaric aciduria; unusual presentation, family studies and dietary management. Eur J Pediatr 1985; 144: 177-181. (Pubitemid 15006998)
    • (1985) European Journal of Pediatrics , vol.144 , Issue.2 , pp. 177-181
    • Stacey, T.E.1    De Sousa, C.2    Tracey, B.M.3
  • 9
    • 34447109475 scopus 로고    scopus 로고
    • 3-Hydroxy-3-Methylglutaryl Coenzyme A Lyase Deficiency with Reversible White Matter Changes after Treatment
    • DOI 10.1016/j.pediatrneurol.2007.02.007, PII S0887899407000926
    • Zaifeiriou DI, Vargiami E, Mayapetek E, Augoustidou-Savvopoulou P, Mitchell GA. 3-Hydroxy-3-methylglutaryl Coenzyme A lyase deficiency with reversible white matter changes after treatment. Pediatr Neurol 2007; 37: 47-50. (Pubitemid 47030784)
    • (2007) Pediatric Neurology , vol.37 , Issue.1 , pp. 47-50
    • Zafeiriou, D.I.1    Vargiami, E.2    Mayapetek, E.3    Augoustidou-Savvopoulou, P.4    Mitchell, G.A.5
  • 11
    • 0001666124 scopus 로고    scopus 로고
    • Inborn errors of ketone body metabolism
    • Scriver CR, Beaudet AL, Sly WS, Valle D (eds.) New York: McGraw-Hill
    • Mitchell GA, Fukao TJC. Inborn errors of ketone body metabolism . In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds.). The Metabolic and Molecular Bases of Inherited Disease, 8th ed. New York: McGraw-Hill, 2001. pp. 2340-2342.
    • (2001) The Metabolic and Molecular Bases of Inherited Disease, 8th Ed , pp. 2340-2342
    • Mitchell, G.A.1    Fukao, T.J.C.2
  • 12
    • 77956061703 scopus 로고    scopus 로고
    • A case of dilated cardiomiopathy associated with 3-hydroxy-3- methylglutaryl-Coenzyme A (HMG CoA) lyase deficiency
    • Leung AA, Chan AK, Ezekowitz JA, Leung AK. A case of dilated cardiomiopathy associated with 3-hydroxy-3-methylglutaryl-Coenzyme A (HMG CoA) lyase deficiency. Case Report Med 2009; 2009: 183125.
    • (2009) Case Report Med , vol.2009 , pp. 183125
    • Leung, A.A.1    Chan, A.K.2    Ezekowitz, J.A.3    Leung, A.K.4
  • 14
    • 39649086269 scopus 로고    scopus 로고
    • Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats
    • Leipnitz G, Seminotti B, Amaral AU, de Bortoli G, Solano A, Schuck PF, et al. Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats. Life Sci 2008; 82: 652-662.
    • (2008) Life Sci , vol.82 , pp. 652-662
    • Leipnitz, G.1    Seminotti, B.2    Amaral, A.U.3    De Bortoli, G.4    Solano, A.5    Schuck, P.F.6
  • 15
    • 67349164287 scopus 로고    scopus 로고
    • Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver
    • Leipnitz G, Seminotti B, Fernandes CG, Amaral AU, Beskow AP, da Silva L de B, et al. Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver. Int J Dev Neurosci 2009; 27: 351-356.
    • (2009) Int J Dev Neurosci , vol.27 , pp. 351-356
    • Leipnitz, G.1    Seminotti, B.2    Fernandes, C.G.3    Amaral, A.U.4    Beskow, A.P.5    De Silva, L.B.6
  • 16
    • 78650171622 scopus 로고    scopus 로고
    • Neurochemical evidence that 3-methylglutaric acid inhibits synaptic Na +, K + -ATPase activity probably through oxidative damage in brain cortex of young rats
    • Ribeiro CA, Hickmann FH, Wajner M. Neurochemical evidence that 3-methylglutaric acid inhibits synaptic Na +, K + -ATPase activity probably through oxidative damage in brain cortex of young rats. Int J Dev Neurosci 2011; 29: 1-7.
    • (2011) Int J Dev Neurosci , vol.29 , pp. 1-7
    • Ribeiro, C.A.1    Hickmann, F.H.2    Wajner, M.3
  • 17
    • 84878512756 scopus 로고    scopus 로고
    • In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase de ficiency induce oxidative stress in striatum of developing rats: A potential pathophysiological mechanism of striatal damage in this disorder
    • Fernandes CG, da Rosa MS, Seminotti B, Pierozan P, Martell RW, Lagranha VL, et al. In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase de ficiency induce oxidative stress in striatum of developing rats: a potential pathophysiological mechanism of striatal damage in this disorder . Mol Genet Metab 2013; 109: 144-153.
    • Mol Genet Metab 2013 , vol.109 , pp. 144-153
    • Fernandes, C.G.1    Da Rosa, M.S.2    Seminotti, B.3    Pierozan, P.4    Martell, R.W.5    Lagranha, V.L.6
  • 20
    • 57949099472 scopus 로고    scopus 로고
    • Selective screening for organic acidemias by urine organic acid GC-MS analysis in brazil: Fifteen-year experience
    • Wajner M, Coelho Dde M, Ingrassia R, de Oliveira AB, Busanello EN, Raymond K, et al. Selective screening for organic acidemias by urine organic acid GC-MS analysis in brazil: fifteen-year experience. Clin Chim Acta 2009; 400: 77-81.
    • (2009) Clin Chim Acta , vol.400 , pp. 77-81
    • Wajner, M.1    Coelho Dde, M.2    Ingrassia, R.3    De Oliveira, A.B.4    Busanello, E.N.5    Raymond, K.6
  • 21
    • 0028239163 scopus 로고
    • Oxidative damage to proteins: Spectrophotometric method for carbonyl assay
    • Reznick AZ, Packer L. Oxidative damage to proteins: spectrophotometric method for carbonyl assay. Methods Enzymol 1994; 233: 357-363.
    • (1994) Methods Enzymol , vol.233 , pp. 357-363
    • Reznick, A.Z.1    Packer, L.2
  • 22
    • 0017184389 scopus 로고
    • A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding
    • Bradford MM. A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding. Anal Biochem. 1976; 72: 248-254.
    • (1976) Anal Biochem. , vol.72 , pp. 248-254
    • Bradford, M.M.1
  • 23
    • 0028143826 scopus 로고
    • Differential susceptibility of plasma proteins to oxidative modification: Examination by western blot immunoassay
    • DOI 10.1016/0891-5849(94)90169-4
    • Shacter E, Williams JA, Lim M, Levine RL. Differential susceptibility of plasma proteins to oxidative modification: examination by western blot immunoassay. Free Radic Biol Med 1994; 17: 429-437. (Pubitemid 24320292)
    • (1994) Free Radical Biology and Medicine , vol.17 , Issue.5 , pp. 429-437
    • Shacter, E.1    Williams, J.A.2    Lim, M.3    Levine, R.L.4
  • 24
    • 0026554428 scopus 로고
    • Evaluation of the probe 2 ', 7 ' -dichloroflurescin as an indicator of reactive oxygen species formation and oxidative stress
    • LeBel CP, Ischiropoulos H, Bondy SC. Evaluation of the probe 2 ', 7 ' -dichloroflurescin as an indicator of reactive oxygen species formation and oxidative stress. Chem Res Toxicol 1992; 5: 347-352.
    • (1992) Chem Res Toxicol , vol.5 , pp. 347-352
    • Lebel, C.P.1    Ischiropoulos, H.2    Bondy, S.C.3
  • 26
    • 0032253227 scopus 로고    scopus 로고
    • Reduced glutathione and glutathione disulfide
    • Browne RW, Armstrong D. Reduced glutathione and glutathione disulfide. Methods Mol Biol 1998; 108: 347-352.
    • (1998) Methods Mol Biol , vol.108 , pp. 347-352
    • Browne, R.W.1    Armstrong, D.2
  • 27
    • 85014905187 scopus 로고
    • Pyrogallol autoxidation
    • Greenwald RA (ed.) Boca Raton, FL: CRC Press
    • Marklund SL. Pyrogallol autoxidation. In: Greenwald RA (ed.). Handbook for Oxygen Radical Research. Boca Raton, FL: CRC Press, 1985. pp. 243-247.
    • (1985) Handbook for Oxygen Radical Research , pp. 243-247
    • Marklund, S.L.1
  • 28
    • 0021318441 scopus 로고
    • Catalase in vitro
    • Aebi H. Catalase in vitro. Methods Enzymol 1994; 105: 121-126.
    • (1994) Methods Enzymol , vol.105 , pp. 121-126
    • Aebi, H.1
  • 29
    • 0019740344 scopus 로고
    • Glutathione peroxidise
    • Wendel A. Glutathione peroxidise. Methods Enzymol 1981; 77: 325-332.
    • (1981) Methods Enzymol , vol.77 , pp. 325-332
    • Wendel, A.1
  • 31
    • 0032471373 scopus 로고    scopus 로고
    • MR imaging and proton spectroscopy in 3-hydroxy-3-methylglutaryl coenzyme a lyase deficiency
    • van der Knaap MS, Bakker HD, Valk J. MR imaging and proton spectroscopy in 3-hydroxy-3-methylglutaryl coenzyme A lyase deficiency. Am J Neuroradiol 1998; 19: 378-382. (Pubitemid 30413812)
    • (1998) American Journal of Neuroradiology , vol.19 , Issue.2 , pp. 378-382
    • Van Der Knaap, M.S.1    Bakker, H.D.2    Valk, J.3
  • 32
    • 33746192561 scopus 로고    scopus 로고
    • Corticospinal Tract Involvement in a Patient With 3-HMG Coenzyme A Lyase Deficiency
    • DOI 10.1016/j.pediatrneurol.2006.01.009, PII S0887899406000804
    • Yylmaz Y, Ozdemir N, Ekinci G, Baykal T, Kocaman C. Corticospinal tract involvement in a patient with 3-HMG coenzyme A lyase deficiency. Pediatr Neurol 2006; 35: 139-141. (Pubitemid 44093981)
    • (2006) Pediatric Neurology , vol.35 , Issue.2 , pp. 139-141
    • Yylmaz, Y.1    Ozdemir, N.2    Ekinci, G.3    Baykal, T.4    Kocaman, C.5
  • 33
  • 34
    • 20444373701 scopus 로고    scopus 로고
    • Proteins in human brain cortex are modified by oxidation, glycoxidation, and lipoxidation: Effects of Alzheimer disease and identification of lipoxidation targets
    • DOI 10.1074/jbc.M502255200
    • Pamplona R, Dalfó E, Ayala V, Bellmunt MJ, Prat J, Ferrer I, Portero-Otín M. Proteins in human brain cortex are modified by oxidation, glycoxidation, and lipoxidation. Effects of Alzheimer disease and identification of lipoxidation targets. J Biol Chem 2005; 280: 21522-21530. (Pubitemid 40805718)
    • (2005) Journal of Biological Chemistry , vol.280 , Issue.22 , pp. 21522-21530
    • Pamplona, R.1    Dalfo, E.2    Ayala, V.3    Bellmunt, M.J.4    Prat, J.5    Ferrer, I.6    Portero-Otin, M.7
  • 36
    • 33846781786 scopus 로고    scopus 로고
    • Reactive oxygen species and antioxidant mechanisms in human tissues and their relation to malignancies
    • DOI 10.1111/j.1600-0463.2007.apm-514.x
    • Karihtala P, Soini Y. Reactive oxygen species and antioxidant mechanisms in human tissues and their relation to malignancies . APMIS 2007; 115: 81-103. (Pubitemid 46208084)
    • (2007) APMIS , vol.115 , Issue.2 , pp. 81-103
    • Karihtala, P.1    Soini, Y.2
  • 37
    • 36048951429 scopus 로고    scopus 로고
    • Measurement of reactive species
    • Halliwell B, Gutteridge JMC (eds.) Oxford: Oxford University Press;
    • Halliwell B, Gutteridge JMC. Measurement of reactive species. In: Halliwell B, Gutteridge JMC (eds.). Free Radicals in Biology and Medicine, 4th ed. Oxford: Oxford University Press; 2007. pp. 268-340.
    • (2007) Free Radicals in Biology and Medicine, 4th Ed , pp. 268-340
    • Halliwell, B.1    Gutteridge, J.M.C.2
  • 38
    • 0032483808 scopus 로고    scopus 로고
    • 2 and tumor necrosis factor-α induce differential binding of the redox-responsive transcription factors AP-1 and NF-κB to the interleukin-8 promoter in endothelial and epithelial cells
    • DOI 10.1074/jbc.273.49.32670
    • Lakshminarayanan V, Drab-Weiss EA, Roebuck KA. H2O2 and tumor necrosis factor-alpha induce differential binding of the redox-responsive transcription factors AP-1 and NFkappaB to the interleukin-8 promoter in endothelial and epithelial cells. J Biol Chem 1998; 273: 32670-32678. (Pubitemid 28557650)
    • (1998) Journal of Biological Chemistry , vol.273 , Issue.49 , pp. 32670-32678
    • Lakshminarayanan, V.1    Drab-Weiss, E.A.2    Roebuck, K.A.3
  • 39
    • 0025948113 scopus 로고
    • The antioxidant responsive element: Activation by oxidative stress and identification of the dna consensus sequence required for functional activity
    • Rushmore TH, Morton MR, Pickett CB. The antioxidant responsive element. Activation by oxidative stress and identification of the DNA consensus sequence required for functional activity. J Biol Chem 1991; 266: 11632-11639. (Pubitemid 21906996)
    • (1991) Journal of Biological Chemistry , vol.266 , Issue.18 , pp. 11632-11639
    • Rushmore, T.H.1    Morton, M.R.2    Pickett, C.B.3
  • 40
    • 0036325318 scopus 로고    scopus 로고
    • Alterations in antioxidant enzymes and oxidative damage in experimental diabetic rat tissues: Effect of vanadate and fenugreek (Trigonella foenum graecum)
    • DOI 10.1023/A:1016103131408
    • Genet S, Kale KR, Baquer NZ. Alterations in antioxidant enzymes and oxidative damage in experimental diabetic rat tissues: effect of vanadate and fenugreek (Trigonella foenum graecum). Mol Cell Biochem 2002; 236: 7-12. (Pubitemid 34851222)
    • (2002) Molecular and Cellular Biochemistry , vol.236 , Issue.1-2 , pp. 7-12
    • Genet, S.1    Kale, R.K.2    Baquer, N.Z.3
  • 41
    • 0037843247 scopus 로고    scopus 로고
    • Chronic cold exposure affects the antioxidant defense system in various rat tissues
    • DOI 10.1016/S0009-8981(03)00171-2
    • Kaushik S, Kauer J. Chronic cold exposure affects the antioxidant defense system in various rat tissues. Clin Chim Acta 2003; 333: 69-77. (Pubitemid 36694828)
    • (2003) Clinica Chimica Acta , vol.333 , Issue.1-2 , pp. 69-77
    • Kaushik, S.1    Kaur, J.2
  • 42
    • 10044296239 scopus 로고    scopus 로고
    • Effects of acute dimethoate administration on antioxidant status of liver and brain of experimental rats
    • DOI 10.1016/j.tox.2004.06.062, PII S0300483X04003865
    • Sharma Y, Bashir S, Irshad M, Gupta SD, Dogra TD. Effects of acute dimethoate administration on antioxidant status of liver and brain of experimental rats. Toxicology 2005; 206: 49-57. (Pubitemid 39601044)
    • (2005) Toxicology , vol.206 , Issue.1 , pp. 49-57
    • Sharma, Y.1    Bashir, S.2    Irshad, M.3    Gupta, S.D.4    Dogra, T.D.5
  • 44
    • 84865617768 scopus 로고    scopus 로고
    • Attenuation of A β -induced neurotoxicity by thymoquinone via inhibition of mitochondrial dysfunction and oxidative stress
    • Khan A, Vaibhav K, Javed H, Khan MM, Tabassum R, Ahmed ME, et al. Attenuation of A β -induced neurotoxicity by thymoquinone via inhibition of mitochondrial dysfunction and oxidative stress. Mol Cell Biochem 2012; 369: 55-65.
    • (2012) Mol Cell Biochem , vol.369 , pp. 55-65
    • Khan, A.1    Vaibhav, K.2    Javed, H.3    Khan, M.M.4    Tabassum, R.5    Ahmed, M.E.6
  • 45
    • 4344571818 scopus 로고    scopus 로고
    • Impact of hypoglycemia and diabetes on CNS: Correlation of mitochondrial oxidative stress with DNA damage
    • DOI 10.1023/B:MCBI.0000026067.08356.13
    • Singh P, Jain A, Kaur G. Impact of hypoglycemia and diabetes on CNS: correlation of mitochondrial oxidative stress with DNA damage. Mol Cell Biochem 2004; 260: 153-159. (Pubitemid 39127583)
    • (2004) Molecular and Cellular Biochemistry , vol.260 , Issue.1 , pp. 153-159
    • Singh, P.1    Jain, A.2    Kaur, G.3
  • 47


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