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Volumn 12, Issue 4, 2003, Pages 569-575

The use of antioxidants in Friedreich's ataxia treatment

Author keywords

Cardiomyopathy; Idebenone; Mitochondria; Rare disease

Indexed keywords

ACONITATE HYDRATASE; ALPHA TOCOPHEROL; ANTIOXIDANT; ASCORBIC ACID; FRATAXIN; IDEBENONE; IRON; PLACEBO; SULFUR; UBIDECARENONE;

EID: 0037398112     PISSN: 13543784     EISSN: None     Source Type: Journal    
DOI: 10.1517/13543784.12.4.569     Document Type: Review
Times cited : (25)

References (34)
  • 1
    • 0034642203 scopus 로고    scopus 로고
    • Oxidative stress in patients with Friedreich ataxia
    • SCHULZ JB, DEHMER T, SCHOLS L et al.: Oxidative stress in patients with Friedreich ataxia. Neurology (2000) 55:1719-1721.
    • (2000) Neurology , vol.55 , pp. 1719-1721
    • Schulz, J.B.1    Dehmer, T.2    Schols, L.3
  • 2
    • 0034778392 scopus 로고    scopus 로고
    • Disabled early recruitment of antioxidant defenses in Friedreich's ataxia
    • CHANTREL-GROUSSARD K, GEROMEL V et al.: Disabled early recruitment of antioxidant defenses in Friedreich's ataxia. Hum. Mol. Genet. (2001) 10:2061-2067.
    • (2001) Hum. Mol. Genet. , vol.10 , pp. 2061-2067
    • Chantrel-Groussard, K.1    Geromel, V.2
  • 3
    • 0036603021 scopus 로고    scopus 로고
    • Friedreich ataxia: A paradigm for mitochondrial diseases
    • PUCCIO H, KOENIG M: Friedreich ataxia: a paradigm for mitochondrial diseases. Curr. Opin. Genet. Dev. (2002) 12:272-277.
    • (2002) Curr. Opin. Genet. Dev. , vol.12 , pp. 272-277
    • Puccio, H.1    Koenig, M.2
  • 4
    • 0033000707 scopus 로고    scopus 로고
    • Quinone analogs prevent enzymes targeted in Friedreich ataxia from iron-induced injury in vitro
    • RUSTIN P, MUNNICH A, ROTIG A: Quinone analogs prevent enzymes targeted in Friedreich ataxia from iron-induced injury in vitro. Biofactors (1999) 9:247-251.
    • (1999) Biofactors , vol.9 , pp. 247-251
    • Rustin, P.1    Munnich, A.2    Rotig, A.3
  • 5
    • 0029821176 scopus 로고    scopus 로고
    • Clinical and genetic abnormalities in patients with Friedreich's ataxia
    • DURR A, COSSEE M, AGID Y et al.: Clinical and genetic abnormalities in patients with Friedreich's ataxia. N. Engl. J. Med. (1996) 335:1169-1175.
    • (1996) N. Engl. J. Med. , vol.335 , pp. 1169-1175
    • Durr, A.1    Cossee, M.2    Agid, Y.3
  • 6
    • 0019782799 scopus 로고
    • Friedreich's ataxia: A clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features
    • HARDING AE: Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features. Brain (1981) 104:589-620.
    • (1981) Brain , vol.104 , pp. 589-620
    • Harding, A.E.1
  • 7
    • 0025129284 scopus 로고
    • The Friedreich ataxia gene is assigned to chromosome 9q13-q21 by mapping of tightly linked markets and shows linkage disequilibrium, with D9S15
    • HANAUER A, CHERY M, FUJITA R, DRIESEL AJ, GILGENKRANTZ S, MANDEL JL. The Friedreich ataxia gene is assigned to chromosome 9q13-q21 by mapping of tightly linked markets and shows linkage disequilibrium, with D9S15. Am. J. Hum. Genet. (1990) 46:133-137.
    • (1990) Am. J. Hum. Genet. , vol.46 , pp. 133-137
    • Hanauer, A.1    Chery, M.2    Fujita, R.3    Driesel, A.J.4    Gilgenkrantz, S.5    Mandel, J.L.6
  • 8
    • 13344270899 scopus 로고    scopus 로고
    • Friedreich's ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion
    • CAMPUZANO V, MONTERMINI L, MOLTO MD et al.: Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science (1996) 271:1423-1427.
    • (1996) Science , vol.271 , pp. 1423-1427
    • Campuzano, V.1    Montermini, L.2    Molto, M.D.3
  • 9
    • 0037131228 scopus 로고    scopus 로고
    • Sticky DNA, a long GAA.GAA. TTC triplex that is formed intramolecularly, in the sequence of intron 1 of the frataxin gene
    • VETCHER AA, NAPIERALA M, IYER RR, CHASTAIN PD, GRIFFITH JD, WELLS RD: Sticky DNA, a long GAA.GAA. TTC triplex that is formed intramolecularly, in the sequence of intron 1 of the frataxin gene. J. Biol. Chem. (2002) 277:39217-39227.
    • (2002) J. Biol. Chem. , vol.277 , pp. 39217-39227
    • Vetcher, A.A.1    Napierala, M.2    Iyer, R.R.3    Chastain, P.D.4    Griffith, J.D.5    Wells, R.D.6
  • 10
    • 0035920101 scopus 로고    scopus 로고
    • Sticky DNA, a self-associated complex formed at long GAA*TTC repeats in intron 1 of the frataxin gene, inhibits transcription
    • SAKAMOTO N, OHSHIMA K, MONTERMINI L, PANDOLFO M, WELLS RD: Sticky DNA, a self-associated complex formed at long GAA*TTC repeats in intron 1 of the frataxin gene, inhibits transcription. J. Biol. Chem. (2001) 276:27171-27177.
    • (2001) J. Biol. Chem. , vol.276 , pp. 27171-27177
    • Sakamoto, N.1    Ohshima, K.2    Montermini, L.3    Pandolfo, M.4    Wells, R.D.5
  • 11
    • 9844222853 scopus 로고    scopus 로고
    • Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes
    • CAMPUZANO V, MONTERMINI L, LUTZ Y et al.: Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes. Hum. Mol. Genet. (1997) 6:1771-1780.
    • (1997) Hum. Mol. Genet. , vol.6 , pp. 1771-1780
    • Campuzano, V.1    Montermini, L.2    Lutz, Y.3
  • 12
    • 0037101845 scopus 로고    scopus 로고
    • The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/ S proteins
    • MUHLENHOFF U, RICHHARDT N, RISTOW M, KISPAL G, LILL R: The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/ S proteins. Hum. Mol. Genet. (2002) 11:2025-2036.
    • (2002) Hum. Mol. Genet. , vol.11 , pp. 2025-2036
    • Muhlenhoff, U.1    Richhardt, N.2    Ristow, M.3    Kispal, G.4    Lill, R.5
  • 13
    • 0036799372 scopus 로고    scopus 로고
    • A non-essential function for yeast frataxin in iron-sulfur cluster assembly
    • DUBY G, FOURY F, RAMAZZOTTI A, HERRMANN J, LUTZ T: A non-essential function for yeast frataxin in iron-sulfur cluster assembly. Hum. Mol. Genet. (2002) 11:2635-2643.
    • (2002) Hum. Mol. Genet. , vol.11 , pp. 2635-2643
    • Duby, G.1    Foury, F.2    Ramazzotti, A.3    Herrmann, J.4    Lutz, T.5
  • 14
    • 0031253821 scopus 로고    scopus 로고
    • Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia
    • ROTIG A, DE LONLAY P, CHRETIEN D et al.: Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia. Nat. Genet. (1997) 17:215-217.
    • (1997) Nat. Genet. , vol.17 , pp. 215-217
    • Rotig, A.1    De Lonlay, P.2    Chretien, D.3
  • 15
    • 0030600134 scopus 로고    scopus 로고
    • Iron-sulphur clusters as genetic regulatory switches: The bifunctional iron regulatory protein-1
    • PARASKEVA E, HENTZE MW: Iron-sulphur clusters as genetic regulatory switches: the bifunctional iron regulatory protein-1. FEBS Lett. (1996) 389:40-43.
    • (1996) FEBS Lett. , vol.389 , pp. 40-43
    • Paraskeva, E.1    Hentze, M.W.2
  • 16
    • 0029758487 scopus 로고    scopus 로고
    • Molecular control of vertebrate iron metabolism: mRNA-based regulatory circuits operated by iron, nitric oxide, and oxidative stress
    • HENTZE MW, KUHN LC: Molecular control of vertebrate iron metabolism: mRNA-based regulatory circuits operated by iron, nitric oxide, and oxidative stress. Proc. Natl. Acad. Sci. USA (1996) 93:8175-8182.
    • (1996) Proc. Natl. Acad. Sci. USA , vol.93 , pp. 8175-8182
    • Hentze, M.W.1    Kuhn, L.C.2
  • 17
    • 0032167632 scopus 로고    scopus 로고
    • Activation of iron regulatory protein-1 by oxidative stress in vitro
    • PANTOPOULOS K, HENTZE MW: Activation of iron regulatory protein-1 by oxidative stress in vitro. Proc. Natl. Acad. Sci. USA (1998) 95:10559-10563.
    • (1998) Proc. Natl. Acad. Sci. USA , vol.95 , pp. 10559-10563
    • Pantopoulos, K.1    Hentze, M.W.2
  • 18
    • 0027948173 scopus 로고
    • Translational control by iron-responsive elements
    • HENTZE MW: Translational control by iron-responsive elements. Adv. Exp. Med. Biol. (1994) 356:119-126.
    • (1994) Adv. Exp. Med. Biol. , vol.356 , pp. 119-126
    • Hentze, M.W.1
  • 19
    • 0242722813 scopus 로고    scopus 로고
    • Frataxin and mitochondrial iron
    • DM Templeton (Ed.) Marcel Dekker, Inc., New York/Basel
    • RUSTIN P: Frataxin and mitochondrial iron. In: Molecular and Cellular Iron Transport. DM Templeton (Ed.) Marcel Dekker, Inc., New York/Basel (2002):255-272.
    • (2002) Molecular and Cellular Iron Transport , pp. 255-272
    • Rustin, P.1
  • 20
    • 0030846021 scopus 로고    scopus 로고
    • Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin
    • BABCOCK M, DE SILVA D, OAKS R et al.: Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin. Science (1997) 276:1709-1712.
    • (1997) Science , vol.276 , pp. 1709-1712
    • Babcock, M.1    De Silva, D.2    Oaks, R.3
  • 21
    • 0032800601 scopus 로고    scopus 로고
    • Low iron concentration and aconitase deficiency in a yeast frataxin homologue deficient strain
    • FOURY F: Low iron concentration and aconitase deficiency in a yeast frataxin homologue deficient strain. FEBS Lett. (1999) 456:281-284.
    • (1999) FEBS Lett. , vol.456 , pp. 281-284
    • Foury, F.1
  • 23
    • 0036252812 scopus 로고    scopus 로고
    • Molecular insights into Friedreich's ataxia and antioxidant-based therapies
    • ROTIG A, SIDI D, MUNNICH A, RUSTIN P: Molecular insights into Friedreich's ataxia and antioxidant-based therapies. Trends Mol. Med. (2002) 8:221-224.
    • (2002) Trends Mol. Med. , vol.8 , pp. 221-224
    • Rotig, A.1    Sidi, D.2    Munnich, A.3    Rustin, P.4
  • 24
    • 0027486590 scopus 로고
    • Subcellular distribution of desferrioxamine and hydroxypyridin-4-one chelators in K562 cells affects chelation of intracellular iron pools
    • PORTER KHJ: Subcellular distribution of desferrioxamine and hydroxypyridin-4-one chelators in K562 cells affects chelation of intracellular iron pools. Br. J. Hematol. (1993) 85:393-400.
    • (1993) Br. J. Hematol. , vol.85 , pp. 393-400
    • Porter, K.H.J.1
  • 25
    • 0036221156 scopus 로고    scopus 로고
    • Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia
    • HAUSSE AO, AGGOUN Y, BONNET D et al.: Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia. Heart (2002) 87:346-349.
    • (2002) Heart , vol.87 , pp. 346-349
    • Hausse, A.O.1    Aggoun, Y.2    Bonnet, D.3
  • 26
    • 0019464939 scopus 로고
    • Vitamin C and iron
    • NIENHUIS A: Vitamin C and iron. N. Engl. J. Med. (1981) 304:170-171.
    • (1981) N. Engl. J. Med. , vol.304 , pp. 170-171
    • Nienhuis, A.1
  • 27
    • 0029042393 scopus 로고
    • Biochemical, physiological and medical aspects of ubiquinone function
    • 1271
    • ERNSTER L, DALLNER G: Biochemical, physiological and medical aspects of ubiquinone function. Biochim. Biophys. Acta (1995) 1271:195-204.
    • (1995) Biochim. Biophys. Acta , pp. 195-204
    • Ernster, L.1    Dallner, G.2
  • 28
    • 0036398010 scopus 로고    scopus 로고
    • Coenzyme Q(10) and idebenone in the therapy of respiratory chain diseases: Rationale and comparative benefits
    • GEROMEL V, DARIN N, CHRETIEN D et al.: Coenzyme Q(10) and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits. Mol. Genet. Metab. (2002) 77:21.
    • (2002) Mol. Genet. Metab. , vol.77 , pp. 21
    • Geromel, V.1    Darin, N.2    Chretien, D.3
  • 29
    • 0034730011 scopus 로고    scopus 로고
    • Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency
    • ROTIG A, APPELKVIST EL, GEROMEL V et al.: Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency. Lancet (2000) 356:391-395.
    • (2000) Lancet , vol.356 , pp. 391-395
    • Rotig, A.1    Appelkvist, E.L.2    Geromel, V.3
  • 30
    • 0037235890 scopus 로고    scopus 로고
    • Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia
    • BUNSE M, BIT-AVRAGIM N, RIEFFLIN A et al.: Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia. Ann. Neurol. (2003) 53:121-123.
    • (2003) Ann. Neurol. , vol.53 , pp. 121-123
    • Bunse, M.1    Bit-Avragim, N.2    Riefflin, A.3
  • 32
    • 0035887745 scopus 로고    scopus 로고
    • Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice
    • SORENSEN L, EKSTRAND M, SILVA JP et al.: late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice. J. Neurosci. (2001) 21:8082-8090.
    • (2001) J. Neurosci. , vol.21 , pp. 8082-8090
    • Sorensen, L.1    Ekstrand, M.2    Silva, J.P.3
  • 33
    • 0036694802 scopus 로고    scopus 로고
    • Friedreich's ataxia: Idebenone treatment in early stage patients
    • ARTUCH R, ARACIL A, MAS A et al.: Friedreich's ataxia: idebenone treatment in early stage patients. Neuropediatrics (2002) 33:190-193.
    • (2002) Neuropediatrics , vol.33 , pp. 190-193
    • Artuch, R.1    Aracil, A.2    Mas, A.3
  • 34
    • 0035020940 scopus 로고    scopus 로고
    • Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia
    • LODI R, HART PE, RAJAGOPALAN B et al.: Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia. Ann. Neurol. (2001) 49:590-596.
    • (2001) Ann. Neurol. , vol.49 , pp. 590-596
    • Lodi, R.1    Hart, P.E.2    Rajagopalan, B.3


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