메뉴 건너뛰기




Volumn 101, Issue 11, 2012, Pages

Patients with organic acidaemias have an altered thiol status

Author keywords

Cysteine; Glutathione; Inborn errors of metabolism; Oxidative stress

Indexed keywords

ACID; CARNITINE; CYSTEINE; GLUCOSE 6 PHOSPHATE DEHYDROGENASE; GLUTATHIONE; GLUTATHIONE PEROXIDASE; GLUTATHIONE REDUCTASE; GLUTATHIONE TRANSFERASE; GLYCINE; METRONIDAZOLE; THIOL;

EID: 84867097606     PISSN: 08035253     EISSN: 16512227     Source Type: Journal    
DOI: 10.1111/j.1651-2227.2012.02799.x     Document Type: Article
Times cited : (23)

References (24)
  • 1
    • 33745098400 scopus 로고    scopus 로고
    • 'Classical' organic acidurias, propionic aciduria, methylmalonic aciduria and isovaleric aciduria: Long-term outcome and effects of expanded newborn screening using tandem mass spectrometry
    • Dionisi-Vici C, Deodato F, Röschinger W, Rhead W, Wilcken B,. 'Classical' organic acidurias, propionic aciduria, methylmalonic aciduria and isovaleric aciduria: long-term outcome and effects of expanded newborn screening using tandem mass spectrometry. J Inherit Metab Dis 2006; 29: 383-9.
    • (2006) J Inherit Metab Dis , vol.29 , pp. 383-389
    • Dionisi-Vici, C.1    Deodato, F.2    Röschinger, W.3    Rhead, W.4    Wilcken, B.5
  • 2
    • 0003237157 scopus 로고    scopus 로고
    • Branched chain organic acidurias
    • Scriver C.R. Beaudet A.L. Sly W.S. Valle D. editors. New York: McGraw-Hill.
    • Sweetman L, Williams JC,. Branched chain organic acidurias. In:, Scriver CR, Beaudet AL, Sly WS, Valle D, editors. The metabolic and molecular bases of inherited disease. New York: McGraw-Hill, 2001.
    • (2001) The Metabolic and Molecular Bases of Inherited Disease
    • Sweetman, L.1    Williams, J.C.2
  • 3
    • 0002911516 scopus 로고    scopus 로고
    • Disorders of propionate and methylmalonate metabolism
    • Scriver C.R. Beaudet A.L. Sly W.S. Valle D. editors. New York: McGraw-Hill.
    • Fenton WA, Gravel RA, Rosenblatt DS,. Disorders of propionate and methylmalonate metabolism. In:, Scriver CR, Beaudet AL, Sly WS, Valle D, editors. The metabolic and molecular bases of inherited disease. New York: McGraw-Hill, 2001.
    • (2001) The Metabolic and Molecular Bases of Inherited Disease
    • Fenton, W.A.1    Gravel, R.A.2    Rosenblatt, D.S.3
  • 4
    • 33751051820 scopus 로고    scopus 로고
    • The aetiology of neurological complications of organic acidaemias - A role for the blood-brain barrier
    • Kölker S, Sauer SW, Surtees RA, Leonard JV,. The aetiology of neurological complications of organic acidaemias-a role for the blood-brain barrier. J Inherit Metab Dis 2006; 29: 701-4.
    • (2006) J Inherit Metab Dis , vol.29 , pp. 701-704
    • Kölker, S.1    Sauer, S.W.2    Surtees, R.A.3    Leonard, J.V.4
  • 5
    • 0034647727 scopus 로고    scopus 로고
    • Methylmalonate administration decreases na+, K+-ATPase activity in cerebral cortex of rats
    • Wyse AT, Streck EL, Barros SV, Brusque AM, Zugno AI, Wajner M,. Methylmalonate administration decreases na+, K+-ATPase activity in cerebral cortex of rats. NeuroReport 2000; 11: 2331-4.
    • (2000) NeuroReport , vol.11 , pp. 2331-2334
    • Wyse, A.T.1    Streck, E.L.2    Barros, S.V.3    Brusque, A.M.4    Zugno, A.I.5    Wajner, M.6
  • 7
    • 0037177804 scopus 로고    scopus 로고
    • Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity
    • Okun JG, Horster F, Farkas LM, Feyh P, Hinz A, Sauer S, et al., Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity. J Biol Chem 2002; 277: 14674-80.
    • (2002) J Biol Chem , vol.277 , pp. 14674-14680
    • Okun, J.G.1    Horster, F.2    Farkas, L.M.3    Feyh, P.4    Hinz, A.5    Sauer, S.6
  • 8
    • 79952317855 scopus 로고    scopus 로고
    • Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia
    • Melo DR, Kowaltowski AJ, Wajner M, Castilho RF,. Mitochondrial energy metabolism in neurodegeneration associated with methylmalonic acidemia. J Bioenerg Biomembr 2011; 43: 39-46.
    • (2011) J Bioenerg Biomembr , vol.43 , pp. 39-46
    • Melo, D.R.1    Kowaltowski, A.J.2    Wajner, M.3    Castilho, R.F.4
  • 9
    • 40749086850 scopus 로고    scopus 로고
    • Neurodegeneration and chronic renal failure in methylmalonic aciduria - A pathophysiological approach
    • Morath MA, Okun JG, Muller IB, Sauer SW, Horster F, Hoffmann GF, et al., Neurodegeneration and chronic renal failure in methylmalonic aciduria-a pathophysiological approach. J Inherit Metab Dis 2008; 31: 35-43.
    • (2008) J Inherit Metab Dis , vol.31 , pp. 35-43
    • Morath, M.A.1    Okun, J.G.2    Muller, I.B.3    Sauer, S.W.4    Horster, F.5    Hoffmann, G.F.6
  • 10
    • 79959606454 scopus 로고    scopus 로고
    • Experimental evidence that methylmalonic acid provokes oxidative damage and compromises antioxidant defenses in nerve terminal and striatum of young rats
    • Fernandes CG, Borges CG, Seminotti B, Amaral AU, Knebel LA, Eichler P, et al., Experimental evidence that methylmalonic acid provokes oxidative damage and compromises antioxidant defenses in nerve terminal and striatum of young rats. Cell Mol Neurobiol 2011; 31: 775-85.
    • (2011) Cell Mol Neurobiol , vol.31 , pp. 775-785
    • Fernandes, C.G.1    Borges, C.G.2    Seminotti, B.3    Amaral, A.U.4    Knebel, L.A.5    Eichler, P.6
  • 12
    • 67749117788 scopus 로고    scopus 로고
    • Profiling of oxidative stress in patients with inborn errors of metabolism
    • McGuire PJ, Parikh A, Diaz GA,. Profiling of oxidative stress in patients with inborn errors of metabolism. Mol Genet Metab 2009; 98: 173-80.
    • (2009) Mol Genet Metab , vol.98 , pp. 173-180
    • McGuire, P.J.1    Parikh, A.2    Diaz, G.A.3
  • 13
    • 84856553552 scopus 로고    scopus 로고
    • Oxidative stress parameters in urine from patients with disorders of propionate metabolism: A beneficial effect of L:-carnitine supplementation
    • Ribas GS, Biancini GB, Mescka C, Wayhs CY, Sitta A, Wajner M, et al., Oxidative stress parameters in urine from patients with disorders of propionate metabolism: a beneficial effect of L:-carnitine supplementation. Cell Mol Neurobiol 2012; 32: 77-82.
    • (2012) Cell Mol Neurobiol , vol.32 , pp. 77-82
    • Ribas, G.S.1    Biancini, G.B.2    Mescka, C.3    Wayhs, C.Y.4    Sitta, A.5    Wajner, M.6
  • 15
    • 0029007943 scopus 로고
    • Glutathione metabolism
    • Meister A,. Glutathione metabolism. Methods Enzymol 1995; 251: 3-7.
    • (1995) Methods Enzymol , vol.251 , pp. 3-7
    • Meister, A.1
  • 16
    • 33750011601 scopus 로고    scopus 로고
    • Mitochondrial glutathione transport: Physiological, pathological and toxicological implications
    • Lash LH,. Mitochondrial glutathione transport: physiological, pathological and toxicological implications. Chem Biol Interact 2006; 163: 54-67.
    • (2006) Chem Biol Interact , vol.163 , pp. 54-67
    • Lash, L.H.1
  • 17
    • 39849110778 scopus 로고    scopus 로고
    • Mechanisms through which sulfur amino acids control protein metabolism and oxidative status
    • Metayer S, Seiliez I, Collin A, Duchene S, Mercier Y, Geraert PA, et al., Mechanisms through which sulfur amino acids control protein metabolism and oxidative status. J Nutr Biochem 2008; 19: 207-15.
    • (2008) J Nutr Biochem , vol.19 , pp. 207-215
    • Metayer, S.1    Seiliez, I.2    Collin, A.3    Duchene, S.4    Mercier, Y.5    Geraert, P.A.6
  • 18
    • 0029033734 scopus 로고
    • Body mass index reference curves for the UK, 1990
    • Cole TJ, Freeman JV, Preece MA,. Body mass index reference curves for the UK, 1990. Arch Dis Child 1995; 73: 17-24.
    • (1995) Arch Dis Child , vol.73 , pp. 17-24
    • Cole, T.J.1    Freeman, J.V.2    Preece, M.A.3
  • 19
    • 84858376962 scopus 로고    scopus 로고
    • Plasma thiol status is altered in children with mitochondrial diseases
    • Salmi H, Leonard JV, Rahman S, Lapatto R,. Plasma thiol status is altered in children with mitochondrial diseases. Scand J Clin Lab Invest 2012; 72: 152-1557.
    • (2012) Scand J Clin Lab Invest , vol.72 , pp. 152-1557
    • Salmi, H.1    Leonard, J.V.2    Rahman, S.3    Lapatto, R.4
  • 21
    • 0036829579 scopus 로고    scopus 로고
    • Redox analysis of human plasma allows separation of pro-oxidant events of aging from decline in antioxidant defenses
    • Jones DP, Mody VC Jr, Carlson JL, Lynn MJ, Sternberg P Jr,. Redox analysis of human plasma allows separation of pro-oxidant events of aging from decline in antioxidant defenses. Free Radic Biol Med 2002; 33: 1290-300.
    • (2002) Free Radic Biol Med , vol.33 , pp. 1290-1300
    • Jones, D.P.1    Mody, Jr.V.C.2    Carlson, J.L.3    Lynn, M.J.4    Sternberg, Jr.P.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.