메뉴 건너뛰기




Volumn 18, Issue 1-4, 2003, Pages 163-171

Friedreich's Ataxia: Disease mechanisms, antioxidant and Coenzyme Q 10 therapy

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLCYSTEINE; ALPHA TOCOPHEROL; ANTIOXIDANT; IDEBENONE; IRON; IRON SULFUR PROTEIN; SELENIUM; UBIDECARENONE;

EID: 0346158441     PISSN: 09516433     EISSN: None     Source Type: Journal    
DOI: 10.1002/biof.5520180219     Document Type: Conference Paper
Times cited : (81)

References (64)
  • 1
    • 0033838364 scopus 로고    scopus 로고
    • Iron-dependent self-assembly of recombinant yeast frataxin: Implications for Friedreich's ataxia
    • J. Adamec, F. Rusnak, W.F. Owen, S. Naylor, L.M. Benson, A.M. Gacy and G. Isaya, Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich's ataxia, Ann J Hum Genet 67 (2000), 549-562.
    • (2000) Ann J Hum Genet , vol.67 , pp. 549-562
    • Adamec, J.1    Rusnak, F.2    Owen, W.F.3    Naylor, S.4    Benson, L.M.5    Gacy, A.M.6    Isaya, G.7
  • 2
    • 0030846021 scopus 로고    scopus 로고
    • Regulation of mitochondrial iron accumulation by Yfhlp, a putative homolog of frataxin
    • M. Babcock, D. de Silva, R. Oaks, S. Davis-Kaplan, S. Jiralerspong, L. Montermini et al., Regulation of mitochondrial iron accumulation by Yfhlp, a putative homolog of frataxin, Science 276 (1997), 1709-1712.
    • (1997) Science , vol.276 , pp. 1709-1712
    • Babcock, M.1    De Silva, D.2    Oaks, R.3    Davis-Kaplan, S.4    Jiralerspong, S.5    Montermini, L.6
  • 3
    • 0026879664 scopus 로고
    • An analysis of the role of coenzyme Q in free radical generation and as an antioxidant
    • R.E. Beyer, An analysis of the role of coenzyme Q in free radical generation and as an antioxidant, Biochem Cell Biol 70 (1992), 390-403.
    • (1992) Biochem Cell Biol , vol.70 , pp. 390-403
    • Beyer, R.E.1
  • 4
    • 0031941447 scopus 로고    scopus 로고
    • The GAA triplet-repeat expansion Friedreich's ataxia interferes with transcription and may be associated with an unusual DNA structure
    • S.I. Bidichandani, T. Ashizawa and P.I. Patel, The GAA triplet-repeat expansion Friedreich's ataxia interferes with transcription and may be associated with an unusual DNA structure, Am J Hum Genet 62 (1998), 111-121.
    • (1998) Am J Hum Genet , vol.62 , pp. 111-121
    • Bidichandani, S.I.1    Ashizawa, T.2    Patel, P.I.3
  • 5
    • 0027872310 scopus 로고
    • Reduced risk of colon cancer with high intake of vitamin E: The Iowa Women's Health Study
    • R.M. Bostick, J.D. Potter, D.R. McKenzie, T.A. Sellers, L.H. Kushi, K.A. Steinmetz et al., Reduced risk of colon cancer with high intake of vitamin E: the Iowa Women's Health Study, Cancer Res 53 (1993), 4230-4237.
    • (1993) Cancer Res , vol.53 , pp. 4230-4237
    • Bostick, R.M.1    Potter, J.D.2    McKenzie, D.R.3    Sellers, T.A.4    Kushi, L.H.5    Steinmetz, K.A.6
  • 7
    • 0037235890 scopus 로고    scopus 로고
    • Cardiac energetics correlates to myocardial hypertrophy in Friedreich's Ataxia
    • M. Bunse, N. Bit-Avragim, A. Riefflin, A. Perrot, O. Schmidt, F.R. Kreuz et al., Cardiac energetics correlates to myocardial hypertrophy in Friedreich's Ataxia, Annn Neurol 53 (2003), 121-123.
    • (2003) Annn Neurol , vol.53 , pp. 121-123
    • Bunse, M.1    Bit-Avragim, N.2    Riefflin, A.3    Perrot, A.4    Schmidt, O.5    Kreuz, F.R.6
  • 8
    • 0023709438 scopus 로고
    • The alpha-tocopherol and phospholipid fatty acid content of rat liver subcellular membranes in vitamin E and selenium deficiency
    • J.L. Buttriss and A.T. Diplock, The alpha-tocopherol and phospholipid fatty acid content of rat liver subcellular membranes in vitamin E and selenium deficiency, Biochim Biophys Acta 963 (1988), 61-69.
    • (1988) Biochim Biophys Acta , vol.963 , pp. 61-69
    • Buttriss, J.L.1    Diplock, A.T.2
  • 9
    • 13344270899 scopus 로고    scopus 로고
    • Friedreich's ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion
    • V. Campuzano, L. Montermini, M.D. Molto, L. Pianese, M. Cossee, F. Cavalcanti et al., Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion, Science 271 (1996), 1423-1427.
    • (1996) Science , vol.271 , pp. 1423-1427
    • Campuzano, V.1    Montermini, L.2    Molto, M.D.3    Pianese, L.4    Cossee, M.5    Cavalcanti, F.6
  • 10
    • 9844222853 scopus 로고    scopus 로고
    • Frataxin is reduced in Friedreich's ataxia patients and is associated with mitochondrial membranes
    • V. Campuzano, L. Montermini, Y. Lutz, L. Cova, C. Hindelang, S. Jiralerspong et al., Frataxin is reduced in Friedreich's ataxia patients and is associated with mitochondrial membranes, Hum Mol Genet 6 (1997), 1771-1780.
    • (1997) Hum Mol Genet , vol.6 , pp. 1771-1780
    • Campuzano, V.1    Montermini, L.2    Lutz, Y.3    Cova, L.4    Hindelang, C.5    Jiralerspong, S.6
  • 11
    • 0036472291 scopus 로고    scopus 로고
    • Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia
    • P. Cavadini, H.A. O'Neill, O. Benada and G. Isaya, Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia, Hum Mol Genet 11 (2002), 217-227.
    • (2002) Hum Mol Genet , vol.11 , pp. 217-227
    • Cavadini, P.1    O'Neill, H.A.2    Benada, O.3    Isaya, G.4
  • 12
    • 0031889483 scopus 로고    scopus 로고
    • Ataxia with isolated vitamin E deficiency: Heterogeneity of mutations and phenotypic variability in a large number of families
    • L. Cavalier, K. Ouahchi, H.J. Kayden, S. Di Donato, L. Reutenauer, J.L. Mandel et al., Ataxia with isolated vitamin E deficiency: heterogeneity of mutations and phenotypic variability in a large number of families, Am J Hum Genet 62 (1998), 301-310.
    • (1998) Am J Hum Genet , vol.62 , pp. 301-310
    • Cavalier, L.1    Ouahchi, K.2    Kayden, H.J.3    Di Donato, S.4    Reutenauer, L.5    Mandel, J.L.6
  • 16
    • 0029821176 scopus 로고    scopus 로고
    • Clinical and genetic abnormalities in patients with Friedreich's ataxia
    • A. Durr, M. Cossee, Y. Agid, V. Campuzano, C. Mignard, C. Penet et al., Clinical and genetic abnormalities in patients with Friedreich's ataxia, N Engl J Med 335 (1996), 1169-1175.
    • (1996) N Engl J Med , vol.335 , pp. 1169-1175
    • Durr, A.1    Cossee, M.2    Agid, Y.3    Campuzano, V.4    Mignard, C.5    Penet, C.6
  • 17
    • 0034642214 scopus 로고    scopus 로고
    • Increased levels of plasma malondialdehyde in Friedreich's ataxia
    • M. Emond, G. Lepage, M. Vanasse and M. Pandolfo, Increased levels of plasma malondialdehyde in Friedreich's ataxia, Neurology 55 (2000), 1752-1753.
    • (2000) Neurology , vol.55 , pp. 1752-1753
    • Emond, M.1    Lepage, G.2    Vanasse, M.3    Pandolfo, M.4
  • 18
    • 0029042393 scopus 로고
    • Biochemical, physiological and medical aspects of ubiquinone function
    • L. Ernster and G. Dallner, Biochemical, physiological and medical aspects of ubiquinone function, Biochim Biophys Acta 1271 (1995), 195-204.
    • (1995) Biochim Biophys Acta , vol.1271 , pp. 195-204
    • Ernster, L.1    Dallner, G.2
  • 19
    • 0029757676 scopus 로고    scopus 로고
    • The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia
    • A. Filla, G. De Michele, F. Cavalcanti, L. Pianese, A. Monticelli, G. Campanella et al., The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia, Am J Hum Genet 59 (1996), 554-560.
    • (1996) Am J Hum Genet , vol.59 , pp. 554-560
    • Filla, A.1    De Michele, G.2    Cavalcanti, F.3    Pianese, L.4    Monticelli, A.5    Campanella, G.6
  • 21
    • 0031567601 scopus 로고    scopus 로고
    • Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria
    • F. Foury and O. Cazzalini, Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria, FEBS Lett 411 (1997), 373-377.
    • (1997) FEBS Lett , vol.411 , pp. 373-377
    • Foury, F.1    Cazzalini, O.2
  • 22
    • 0032800601 scopus 로고    scopus 로고
    • Low iron concentration and aconitase deficiency in a yeast frataxin homologue deficient strain
    • F. Foury, Low iron concentration and aconitase deficiency in a yeast frataxin homologue deficient strain, FEBS Lett 456 (1999), 281-284.
    • (1999) FEBS Lett , vol.456 , pp. 281-284
    • Foury, F.1
  • 23
    • 0036179037 scopus 로고    scopus 로고
    • Rationale for clinical trials of selenium as an antioxidant for the treatment of the cardiomyopathy of Friedreich's ataxia
    • M.J. Fryer, Rationale for clinical trials of selenium as an antioxidant for the treatment of the cardiomyopathy of Friedreich's ataxia, Med. Hypotheses 58 (2002), 127-132.
    • (2002) Med. Hypotheses , vol.58 , pp. 127-132
    • Fryer, M.J.1
  • 24
    • 0035726073 scopus 로고    scopus 로고
    • Effect of vitamin E supplementation in patients with ataxia with vitamin E deficiency
    • S. Gabsi, N. Gouider-Khouja, S. Belal, M. Fki, M. Kefi, I. Turki et al., Effect of vitamin E supplementation in patients with ataxia with vitamin E deficiency, Eur J Neurol 8 (2001), 477-481.
    • (2001) Eur J Neurol , vol.8 , pp. 477-481
    • Gabsi, S.1    Gouider-Khouja, N.2    Belal, S.3    Fki, M.4    Kefi, M.5    Turki, I.6
  • 25
    • 0031593616 scopus 로고    scopus 로고
    • Sustained efficacy and safety of idebenone in the treatment of Alzheimer's disease: Update on a 2-year double-blind multicentre study
    • H. Gutzmann and D. Hadler, Sustained efficacy and safety of idebenone in the treatment of Alzheimer's disease: update on a 2-year double-blind multicentre study, J Neural Transm Suppl 54 (1998), 301-310.
    • (1998) J Neural Transm Suppl , vol.54 , pp. 301-310
    • Gutzmann, H.1    Hadler, D.2
  • 26
    • 0019782799 scopus 로고
    • Friedreich's ataxia: A clinical and genetic study of 90 families with an analysis of early diagnosis criteria and intrafamilial clustering of clinical features
    • A.E. Harding, Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnosis criteria and intrafamilial clustering of clinical features, Brain 104 (1981), 589-620.
    • (1981) Brain , vol.104 , pp. 589-620
    • Harding, A.E.1
  • 27
    • 0024997609 scopus 로고
    • 1-Methyl-4-phenylpyridinium (MPP+) induces NADH-dependent superoxide formation and enhances NADH-dependent lipid peroxidation in bovine heart submitochondrial particles
    • E. Hasegawa, K. Takeshige, T. Oishi, Y. Murai and S. Minakami, 1-Methyl-4-phenylpyridinium (MPP+) induces NADH-dependent superoxide formation and enhances NADH-dependent lipid peroxidation in bovine heart submitochondrial particles, Biochem Biophys Res Commun 170 (1990), 1049-1055.
    • (1990) Biochem Biophys Res Commun , vol.170 , pp. 1049-1055
    • Hasegawa, E.1    Takeshige, K.2    Oishi, T.3    Murai, Y.4    Minakami, S.5
  • 28
    • 0027960215 scopus 로고
    • Superoxide and peroxynitrite inactivate aconitase, but nitric oxide does not
    • A. Hausalden and I. Fridovich, Superoxide and peroxynitrite inactivate aconitase, but nitric oxide does not, J Biol Chem 269 (1994), 29405-29408.
    • (1994) J Biol Chem , vol.269 , pp. 29405-29408
    • Hausalden, A.1    Fridovich, I.2
  • 29
    • 0036221156 scopus 로고    scopus 로고
    • Iebenone and reduced cardiac hypertrophy in Friedreich's ataxia
    • A.O. Hausse, Y. Aggoun, D. Bonnet, D. Sidi, A. Munnich, A. Rotig et al., Iebenone and reduced cardiac hypertrophy in Friedreich's ataxia, Heart 87 (2002), 346-349.
    • (2002) Heart , vol.87 , pp. 346-349
    • Hausse, A.O.1    Aggoun, Y.2    Bonnet, D.3    Sidi, D.4    Munnich, A.5    Rotig, A.6
  • 30
    • 0035504444 scopus 로고    scopus 로고
    • The phylogenetic distribution of frataxin indicates a role in iron-sulphur cluster protein assembly
    • M.A. Huynen, B. Snel, P. Bork and T.J. Gibson, The phylogenetic distribution of frataxin indicates a role in iron-sulphur cluster protein assembly, Hum Mol Genet 10 (2001), 2463-2468.
    • (2001) Hum Mol Genet , vol.10 , pp. 2463-2468
    • Huynen, M.A.1    Snel, B.2    Bork, P.3    Gibson, T.J.4
  • 32
    • 0035976855 scopus 로고    scopus 로고
    • Manganese superoxide dismutase induction by iron is impaired in Friedreich's ataxia cells
    • S. Jiralerspong, B. Ge, T.J. Hudson and M. Pandolfo, Manganese superoxide dismutase induction by iron is impaired in Friedreich's ataxia cells, FEBS Lett 509 (2001), 101-105.
    • (2001) FEBS Lett , vol.509 , pp. 101-105
    • Jiralerspong, S.1    Ge, B.2    Hudson, T.J.3    Pandolfo, M.4
  • 33
    • 0027654218 scopus 로고
    • Quantitative analysis by 31P magnetic resonance spectroscopy of abnormal mitochondrial oxidation in skeletal muscle during recovery from exercise
    • G.J. Kemp, D.J. Taylor, C.H. Thompson, L.J. Hands, B. Rajagopalan, P. Styles et al., Quantitative analysis by 31P magnetic resonance spectroscopy of abnormal mitochondrial oxidation in skeletal muscle during recovery from exercise, NMR Biomed 6 (1993), 302-310.
    • (1993) NMR Biomed , vol.6 , pp. 302-310
    • Kemp, G.J.1    Taylor, D.J.2    Thompson, C.H.3    Hands, L.J.4    Rajagopalan, B.5    Styles, P.6
  • 34
    • 0033565665 scopus 로고    scopus 로고
    • The mitochondrial proteins Atm1p and Nfs1p are essential for biogenesis of cytosolic Fe/S proteins
    • G. Kispal, P. Csere, C. Prohl, and R. Lill, The mitochondrial proteins Atm1p and Nfs1p are essential for biogenesis of cytosolic Fe/S proteins, EMBO J 18 (1999), 3981-3989.
    • (1999) EMBO J , vol.18 , pp. 3981-3989
    • Kispal, G.1    Csere, P.2    Prohl, C.3    Lill, R.4
  • 35
    • 0030813487 scopus 로고    scopus 로고
    • Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin
    • H. Koutnikova, V. Campuzano, F. Foury, P. Dollé, O. Cazzalini and M. Koenig, Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin, Nature Genetics 16 (1997), 345-351.
    • (1997) Nature Genetics , vol.16 , pp. 345-351
    • Koutnikova, H.1    Campuzano, V.2    Foury, F.3    Dollé, P.4    Cazzalini, O.5    Koenig, M.6
  • 36
    • 0001787556 scopus 로고
    • Cardiac iron deposits in Friedreich's ataxia
    • R. Lechtenberg, ed., Marcel Dekker, New York
    • J.B. Lamarche, D. Shapcott, M. Cote and B. Lemieux, Cardiac iron deposits in Friedreich's ataxia, in: Handbook of cerebellar diseases, R. Lechtenberg, ed., Marcel Dekker, New York, 1993, pp. 453-456.
    • (1993) Handbook of Cerebellar Diseases , pp. 453-456
    • Lamarche, J.B.1    Shapcott, D.2    Cote, M.3    Lemieux, B.4
  • 38
    • 0032722872 scopus 로고    scopus 로고
    • Yeast mitochondrial protein, Nfs1p, coordinately regulates iron-sulfur cluster proteins, cellular iron uptake, and iron distribution
    • J. Li, M. Kogan, S.A. Knight, D. Pain and A. Dancis, Yeast mitochondrial protein, Nfs1p, coordinately regulates iron-sulfur cluster proteins, cellular iron uptake, and iron distribution, J Biol Chem 274 (1999), 33025-33034.
    • (1999) J Biol Chem , vol.274 , pp. 33025-33034
    • Li, J.1    Kogan, M.2    Knight, S.A.3    Pain, D.4    Dancis, A.5
  • 39
    • 0032746009 scopus 로고    scopus 로고
    • The essential role of mitochondria in the biogenesis of cellular iron-sulfur proteins
    • R. Lill, K. Diekert, A. Kaut, H. Lange, W. Pelzer, C. Prohl et al., The essential role of mitochondria in the biogenesis of cellular iron-sulfur proteins, Biol Chem 380 (1999), 1157-1166.
    • (1999) Biol Chem , vol.380 , pp. 1157-1166
    • Lill, R.1    Diekert, K.2    Kaut, A.3    Lange, H.4    Pelzer, W.5    Prohl, C.6
  • 41
    • 0034839688 scopus 로고    scopus 로고
    • Cardiac energetics are abnormal in Friedreich's ataxia patients in the absence of cardiac dysfunction and hypertrophy. An in vivo 31P magnetic resonance spectroscopy study is abnormal
    • R. Lodi, B. Rajagopalan, A.M. Blamire, J.M. Cooper, C.H. Davies, J.L. Bradley et al., Cardiac energetics are abnormal in Friedreich's ataxia patients in the absence of cardiac dysfunction and hypertrophy. An in vivo 31P magnetic resonance spectroscopy study is abnormal, Cardiovascular Res 52 (2001), 111-119.
    • (2001) Cardiovascular Res , vol.52 , pp. 111-119
    • Lodi, R.1    Rajagopalan, B.2    Blamire, A.M.3    Cooper, J.M.4    Davies, C.H.5    Bradley, J.L.6
  • 42
    • 0035020940 scopus 로고    scopus 로고
    • Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's Ataxia
    • R. Lodi, B. Rajagopalan, P.E. Hart, A.M. Blamire, J.G. Crilley, J.L. Bradley et al., Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's Ataxia, Ann Neurol 49 (2001), 590-596.
    • (2001) Ann Neurol , vol.49 , pp. 590-596
    • Lodi, R.1    Rajagopalan, B.2    Hart, P.E.3    Blamire, A.M.4    Crilley, J.G.5    Bradley, J.L.6
  • 43
    • 0032555066 scopus 로고    scopus 로고
    • 10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects
    • 10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects, Proc Natl Acad Sci USA 95 (1998), 8892-8897.
    • (1998) Proc Natl Acad Sci USA , vol.95 , pp. 8892-8897
    • Matthews, R.T.1    Yang, L.2    Browne, S.3    Baik, M.4    Beal, M.F.5
  • 45
    • 0031885039 scopus 로고    scopus 로고
    • Antioxidant properties of 2,3-dimethoxy-5-methyl-6-(10-hydroxydecyl)-1,4- benzoquinone (idebenone)
    • A. Mordente, G.E. Martorana, G. Minotti and B. Giardina, Antioxidant properties of 2,3-dimethoxy-5-methyl-6-(10-hydroxydecyl)-1,4-benzoquinone (idebenone), Chem Res Toxicol 11 (1998), 54-63.
    • (1998) Chem Res Toxicol , vol.11 , pp. 54-63
    • Mordente, A.1    Martorana, G.E.2    Minotti, G.3    Giardina, B.4
  • 46
    • 0024501895 scopus 로고
    • Brain distribution of idebenone and its effect on local cerebral glucose utilization in rats
    • Y. Nagai, K. Yoshida, S. Narumi, S. Tanayama and A. Nagaoka, Brain distribution of idebenone and its effect on local cerebral glucose utilization in rats, Arch Gerontol Geriatr 8 (1989), 257-272.
    • (1989) Arch Gerontol Geriatr , vol.8 , pp. 257-272
    • Nagai, Y.1    Yoshida, K.2    Narumi, S.3    Tanayama, S.4    Nagaoka, A.5
  • 49
    • 0035138072 scopus 로고    scopus 로고
    • Mouse models for Friedreich's ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits
    • H. Puccio, D. Simon, M. Cessée, P. Criqui-Filipe, F. Tiziano, J. Melki et al., Mouse models for Friedreich's ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits, Nature Gen 27 (2001), 181-186.
    • (2001) Nature Gen , vol.27 , pp. 181-186
    • Puccio, H.1    Simon, D.2    Cessée, M.3    Criqui-Filipe, P.4    Tiziano, F.5    Melki, J.6
  • 51
    • 0026666969 scopus 로고
    • Tissue concentrations of coenzyme Q10 in the rat following its oral and intraperitoneal administration
    • S. Reahal and J. Wrigglesworth, Tissue concentrations of coenzyme Q10 in the rat following its oral and intraperitoneal administration, Drug Metab Dispos 20 (1992), 423-427.
    • (1992) Drug Metab Dispos , vol.20 , pp. 423-427
    • Reahal, S.1    Wrigglesworth, J.2
  • 52
    • 0035978474 scopus 로고    scopus 로고
    • Development of potential iron chelators for the treatment of Friedreich's ataxia: Ligands that mobilise mitochondrial iron
    • D.R. Richardson, C. Mouralian, P. Ponka and E. Becker, Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilise mitochondrial iron, Biochim et Biophys Acta 1536 (2001), 133-140.
    • (2001) Biochim et Biophys Acta , vol.1536 , pp. 133-140
    • Richardson, D.R.1    Mouralian, C.2    Ponka, P.3    Becker, E.4
  • 53
    • 0031253821 scopus 로고    scopus 로고
    • Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich's ataxia
    • A. Rotig, P. de Lonlay, D. Chretien, F. Foury, M. Koenig, D. Sidi et al., Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich's ataxia, Nature Genetics 17 (1997), 215-217.
    • (1997) Nature Genetics , vol.17 , pp. 215-217
    • Rotig, A.1    De Lonlay, P.2    Chretien, D.3    Foury, F.4    Koenig, M.5    Sidi, D.6
  • 56
    • 0031711714 scopus 로고    scopus 로고
    • DATATOP: A decade of neuroprotective inquiry. Parkinson Study Group. Deprenyl and tocopherol antioxidative therapy of Parkinsonism
    • I. Shoulson, DATATOP: a decade of neuroprotective inquiry. Parkinson Study Group. Deprenyl and tocopherol antioxidative therapy of Parkinsonism, Ann Neurol 44 (1998), S160-166.
    • (1998) Ann Neurol , vol.44
    • Shoulson, I.1
  • 58
    • 0029925181 scopus 로고    scopus 로고
    • Randomised controlled trial of vitamin E in patients with coronary disease: Cambridge Heart Antioxidant Study (CHAOS)
    • N.G. Stephens, A. Parsons, P.M. Schofield, F. Kelly, K. Cheeseman and M.J. Mitchinson, Randomised controlled trial of vitamin E in patients with coronary disease: Cambridge Heart Antioxidant Study (CHAOS), Lancet 347 (1996), 781-786.
    • (1996) Lancet , vol.347 , pp. 781-786
    • Stephens, N.G.1    Parsons, A.2    Schofield, P.M.3    Kelly, F.4    Cheeseman, K.5    Mitchinson, M.J.6
  • 59
    • 0033982887 scopus 로고    scopus 로고
    • Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse
    • S.J. Tabrizi, J. Workman, P.E. Hart, L. Mangiarini, A. Mahal, G. Bates et al., Mitochondrial dysfunction and free radical damage in the Huntington R6/ 2 transgenic mouse, Ann Neurol 47 (2000), 80-86.
    • (2000) Ann Neurol , vol.47 , pp. 80-86
    • Tabrizi, S.J.1    Workman, J.2    Hart, P.E.3    Mangiarini, L.4    Mahal, A.5    Bates, G.6
  • 60
    • 0027739829 scopus 로고
    • Myopathy in vitamin E deficient rats: Muscle fibre necrosis associated with disturbances of mitochondrial function
    • P.K. Thomas, J.M. Cooper, R.H. King, J.M. Workman, A.H. Schapira, M.A. Goss-Sampson et al., Myopathy in vitamin E deficient rats: muscle fibre necrosis associated with disturbances of mitochondrial function, J Anat 183 (1993), 451-461.
    • (1993) J Anat , vol.183 , pp. 451-461
    • Thomas, P.K.1    Cooper, J.M.2    King, R.H.3    Workman, J.M.4    Schapira, A.H.5    Goss-Sampson, M.A.6
  • 62
    • 0031903392 scopus 로고    scopus 로고
    • Normal serum iron and ferritin concentrations in patients with Friedreich's ataxia
    • R.B. Wilson, D.R. Lynch and K.H. Fischbeck, Normal serum iron and ferritin concentrations in patients with Friedreich's ataxia, Ann Neurol 44 (1998), 132-134.
    • (1998) Ann Neurol , vol.44 , pp. 132-134
    • Wilson, R.B.1    Lynch, D.R.2    Fischbeck, K.H.3
  • 63
    • 0033054177 scopus 로고    scopus 로고
    • The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis
    • A. Wong, J. Yang, P. Cavadini, C. Gellera, B. Lonnerdal, F. Taroni et al., The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis, Hum Mol Genet 8 (1999), 425-430.
    • (1999) Hum Mol Genet , vol.8 , pp. 425-430
    • Wong, A.1    Yang, J.2    Cavadini, P.3    Gellera, C.4    Lonnerdal, B.5    Taroni, F.6
  • 64
    • 0028847947 scopus 로고
    • Uptake of dietary coenzyme Q supplement is limited in rats
    • Y. Zhang, F. Aberg, E.L. Appelkvist, G. Dallner and L. Ernster, Uptake of dietary coenzyme Q supplement is limited in rats, J Nutr 125 (1995), 446-453.
    • (1995) J Nutr , vol.125 , pp. 446-453
    • Zhang, Y.1    Aberg, F.2    Appelkvist, E.L.3    Dallner, G.4    Ernster, L.5


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.