-
1
-
-
33947601659
-
Redox regulation of cellular stress response in aging and neurodegenerative disorders: Role of vitagenes
-
Calabrese V et al. (2007) Redox regulation of cellular stress response in aging and neurodegenerative disorders: Role of vitagenes. Neurochem Res 32: 757-773
-
(2007)
Neurochem Res
, vol.32
, pp. 757-773
-
-
Calabrese, V.1
-
2
-
-
33646675336
-
Redox modulation of heat shook protein expression by acetylcamitine in aging brain: Relationship to antioxidant status and mitochondrial function
-
Calabrese V et al. (2006) Redox modulation of heat shook protein expression by acetylcamitine in aging brain: Relationship to antioxidant status and mitochondrial function. Antioxid Redox Signal 8: 404-416
-
(2006)
Antioxid Redox Signal
, vol.8
, pp. 404-416
-
-
Calabrese, V.1
-
3
-
-
37649015008
-
Friedreich ataxia
-
Eds Wells RD and Ashizawa T San Diego: Academic Press
-
Pandolfo M (2006) Friedreich ataxia. In Genetrc Intabilities and Neurological Diseases, edn 2 77-298 (Eds Wells RD and Ashizawa T) San Diego: Academic Press
-
(2006)
Genetrc Intabilities and Neurological Diseases, edn
, vol.2
, pp. 77-298
-
-
Pandolfo, M.1
-
4
-
-
13344270899
-
Friedreich ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion
-
Campuzano V et al. (1996) Friedreich ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion. Science 271:1423-1427
-
(1996)
Science
, vol.271
, pp. 1423-1427
-
-
Campuzano, V.1
-
5
-
-
0033120042
-
Sticky DNA: Self-association properties of long GAA.TTC repeats in R.R.Y triplex structures from Friedreidh's ataxia
-
Sakamoto N et al. (1999) Sticky DNA: Self-association properties of long GAA.TTC repeats in R.R.Y triplex structures from Friedreidh's ataxia. Mol Cell 3; 465-475
-
(1999)
Mol Cell
, vol.3
, pp. 465-475
-
-
Sakamoto, N.1
-
6
-
-
33748778745
-
Histone deacetylase inhibitors reverse gene silendng in Friedreich's ataxia
-
Herman D et al. (2006) Histone deacetylase inhibitors reverse gene silendng in Friedreich's ataxia. Nat Chem Biol 2: 551-558
-
(2006)
Nat Chem Biol
, vol.2
, pp. 551-558
-
-
Herman, D.1
-
7
-
-
0030846021
-
Regulation of mitochondrial iron accumulation by Yfh1, a putative homolog of frataxin
-
Babcock M et al. (1997) Regulation of mitochondrial iron accumulation by Yfh1, a putative homolog of frataxin. Science 276: 1709-1712
-
(1997)
Science
, vol.276
, pp. 1709-1712
-
-
Babcock, M.1
-
8
-
-
0034192352
-
Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation
-
Cossée M et al. (2000) Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation. Hum Mol Genet 9: 1219-1226
-
(2000)
Hum Mol Genet
, vol.9
, pp. 1219-1226
-
-
Cossée, M.1
-
9
-
-
0035138072
-
Mouse models for Friedrech ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits
-
Puccio H et al. (2001) Mouse models for Friedrech ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits. Nat Genet 27: 181-618
-
(2001)
Nat Genet
, vol.27
, pp. 181-618
-
-
Puccio, H.1
-
10
-
-
84882301955
-
Animal models of Friedreich ataxia
-
Ed LeDoux M London: Elsevier Academic Press
-
Pandolfo M (2006) Animal models of Friedreich ataxia. In Animal Models of Movement Disorders, 649-657 (Ed LeDoux M) London: Elsevier Academic Press
-
(2006)
Animal Models of Movement Disorders
, pp. 649-657
-
-
Pandolfo, M.1
-
11
-
-
17144378216
-
Iron-sulphur cluster biogenesis and mitochondrial iron homeostasis
-
Rouault TA and Tong WH (2005) Iron-sulphur cluster biogenesis and mitochondrial iron homeostasis. Nat Rev Mol Cell Biol 6: 345-351
-
(2005)
Nat Rev Mol Cell Biol
, vol.6
, pp. 345-351
-
-
Rouault, T.A.1
Tong, W.H.2
-
12
-
-
35449008450
-
Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues
-
doi:10.1093/hmg/ddm163
-
Martelli A et al. (2007) Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues. Hum Mol Genet [doi:10.1093/hmg/ddm163]
-
(2007)
Hum Mol Genet
-
-
Martelli, A.1
-
13
-
-
0037613459
-
Iron-sulfur cluster biosynthesis: Characterization of frataxin as an iron donor for assembly 9f [2Fe-2S] clusters in ISU-type proteins
-
Yoon T and Cowan JA (2003) Iron-sulfur cluster biosynthesis: characterization of frataxin as an iron donor for assembly 9f [2Fe-2S] clusters in ISU-type proteins. J Am Chem Soc 125: 6078-6084
-
(2003)
J Am Chem Soc
, vol.125
, pp. 6078-6084
-
-
Yoon, T.1
Cowan, J.A.2
-
14
-
-
0033582421
-
The yeast frataxin homologue mediates mitochondrial iron efflux: Evidence for a mitochondrial iron cycle
-
Radisky DC et al. (1999) The yeast frataxin homologue mediates mitochondrial iron efflux: Evidence for a mitochondrial iron cycle. J Biol Chem 274: 4497-4499
-
(1999)
J Biol Chem
, vol.274
, pp. 4497-4499
-
-
Radisky, D.C.1
-
15
-
-
3142667831
-
Transcription of the yeast-iron regulon does not respond directly to iron but rather to iron-sulfur cluster biosynthesis
-
Chen OS et al. (2004) Transcription of the yeast-iron regulon does not respond directly to iron but rather to iron-sulfur cluster biosynthesis. J Biol Chem 279: 29513-29518
-
(2004)
J Biol Chem
, vol.279
, pp. 29513-29518
-
-
Chen, O.S.1
-
16
-
-
0033054177
-
The Friedreich ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis
-
Wong A et al. (1999) The Friedreich ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis. Hum Mol Genet 8 425-430
-
(1999)
Hum Mol Genet
, vol.8
, pp. 425-430
-
-
Wong, A.1
-
17
-
-
34247188143
-
Oxidative stress and protease dysfunction in the yeast model of Friedreich ataxia
-
Bulteau AL et al. (2007) Oxidative stress and protease dysfunction in the yeast model of Friedreich ataxia. Free Radic Biol Med 42 1561-1570
-
(2007)
Free Radic Biol Med
, vol.42
, pp. 1561-1570
-
-
Bulteau, A.L.1
-
18
-
-
0035445849
-
Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm
-
Santos M et al. (2001) Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm. Hum Mol Genet 10: 1935-1944
-
(2001)
Hum Mol Genet
, vol.10
, pp. 1935-1944
-
-
Santos, M.1
-
19
-
-
0036848373
-
Up-regulation of c-Jun N-terminal kinase pathway in Friedreich's ataxia cells
-
Pianese L et al. (2002) Up-regulation of c-Jun N-terminal kinase pathway in Friedreich's ataxia cells. Hum Mol Genet 11: 2989-2996
-
(2002)
Hum Mol Genet
, vol.11
, pp. 2989-2996
-
-
Pianese, L.1
-
20
-
-
0035976855
-
Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells
-
Jiralerspong S et al. (2001) Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells. FEBS Letters 509: 101-105
-
(2001)
FEBS Letters
, vol.509
, pp. 101-105
-
-
Jiralerspong, S.1
-
21
-
-
0034778392
-
Disabled early recruitment of antioxidant defenses in Friedreich's ataxia
-
Chantrel-Groussard K et al. (2001) Disabled early recruitment of antioxidant defenses in Friedreich's ataxia. Hum Mol Genet 10 2061-2067
-
(2001)
Hum Mol Genet
, vol.10
, pp. 2061-2067
-
-
Chantrel-Groussard, K.1
-
22
-
-
0034642214
-
Increased levels of plasma malondialdehyde in Friedreich ataxia
-
Emond M et al. (2000) Increased levels of plasma malondialdehyde in Friedreich ataxia. Neurology 55: 1752-1753
-
(2000)
Neurology
, vol.55
, pp. 1752-1753
-
-
Emond, M.1
-
23
-
-
0034642203
-
Oxidative stress in patients with Friedreich ataxia
-
Schulz JB et al. (2000) Oxidative stress in patients with Friedreich ataxia. Neurology 55: 1719-1721
-
(2000)
Neurology
, vol.55
, pp. 1719-1721
-
-
Schulz, J.B.1
-
24
-
-
0036245640
-
Antioxidant enzymes in blood of patients with Friedreich's ataxia
-
Tozzi G et al. (2002) Antioxidant enzymes in blood of patients with Friedreich's ataxia. Arch Dis Child 86: 376-379
-
(2002)
Arch Dis Child
, vol.86
, pp. 376-379
-
-
Tozzi, G.1
-
25
-
-
14044273058
-
Friedreich ataxia: The oxidative stress paradox
-
Seznec H et al. (2005) Friedreich ataxia: The oxidative stress paradox. Hum Mol Genet 14: 463-474
-
(2005)
Hum Mol Genet
, vol.14
, pp. 463-474
-
-
Seznec, H.1
-
26
-
-
0030939011
-
International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neuropharmacology
-
Trouillas P et al. (1997) International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neuropharmacology J Neurol Sci 145:205-211
-
(1997)
J Neurol Sci
, vol.145
, pp. 205-211
-
-
Trouillas, P.1
-
27
-
-
16844371342
-
Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale
-
Subramony SH et al. (2005) Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale. Neurology 64: 1261-1262
-
(2005)
Neurology
, vol.64
, pp. 1261-1262
-
-
Subramony, S.H.1
-
28
-
-
33745677486
-
Scale for the assessment and rating of ataxia: Development of a new clinical scale
-
Schmitz-Hubsch T et al. (2006) Scale for the assessment and rating of ataxia: Development of a new clinical scale. Neurology 66 1717-1720
-
(2006)
Neurology
, vol.66
, pp. 1717-1720
-
-
Schmitz-Hubsch, T.1
-
29
-
-
0033613262
-
Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia
-
Lodi R et al. (1999) Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia. Proc Natl Aced Sci USA 96: 11492-11495
-
(1999)
Proc Natl Aced Sci USA
, vol.96
, pp. 11492-11495
-
-
Lodi, R.1
-
30
-
-
20144389286
-
Antioxidant treatment of patients with Friedreich ataxia: Four-year follow-up
-
Hart PE et al. (2005) Antioxidant treatment of patients with Friedreich ataxia: Four-year follow-up. Arch Neurol 62 621-626
-
(2005)
Arch Neurol
, vol.62
, pp. 621-626
-
-
Hart, P.E.1
-
31
-
-
0035036258
-
Therapeutic potential of N-acetylcysteine in age-related neurodegenerative mitochondrial diseases
-
Banaclocha MM (2001) Therapeutic potential of N-acetylcysteine in age-related neurodegenerative mitochondrial diseases. Med Hypotheses 56: 472-477
-
(2001)
Med Hypotheses
, vol.56
, pp. 472-477
-
-
Banaclocha, M.M.1
-
32
-
-
0021859645
-
Stimulation of the respiratory and phosphorylating activities in rat brain mitochondria by idebenone (CV-2619), a new agent improving cerebral metabolism
-
Sugiyama Y and Fujita T (1985) Stimulation of the respiratory and phosphorylating activities in rat brain mitochondria by idebenone (CV-2619), a new agent improving cerebral metabolism. FEBS Letters 184: 48-51
-
(1985)
FEBS Letters
, vol.184
, pp. 48-51
-
-
Sugiyama, Y.1
Fujita, T.2
-
33
-
-
1642264124
-
Quinone analogues regulate mitochondrial substrate competitive oxidation
-
Brière JJ et al. (2004) Quinone analogues regulate mitochondrial substrate competitive oxidation.. Biochem Biophys Res Comm 316:1138-1142
-
(2004)
Biochem Biophys Res Comm
, vol.316
, pp. 1138-1142
-
-
Brière, J.J.1
-
34
-
-
0022400051
-
Effects of idebenone (CV-2169) and its metabolites on respiratory activity and lipid peroxidation in brain mitochondria from rats and dogs
-
Sugiyama Y et al. (1985) Effects of idebenone (CV-2169) and its metabolites on respiratory activity and lipid peroxidation in brain mitochondria from rats and dogs. J Pharmacobiodyn 8: 1006-1017
-
(1985)
J Pharmacobiodyn
, vol.8
, pp. 1006-1017
-
-
Sugiyama, Y.1
-
35
-
-
0030758077
-
A controlled study of-2 doses of idebenone in the treatment of Alzheimer's disease
-
Weyer G et al. (1997) A controlled study of-2 doses of idebenone in the treatment of Alzheimer's disease. Neuropsychobiology 36 73-82
-
(1997)
Neuropsychobiology
, vol.36
, pp. 73-82
-
-
Weyer, G.1
-
36
-
-
0344738681
-
Idebenone treatment fails to slow cognitive decline in Alzheimer's disease
-
Thai LJ et al. (2003) Idebenone treatment fails to slow cognitive decline in Alzheimer's disease. Neurology 61: 1498-1502
-
(2003)
Neurology
, vol.61
, pp. 1498-1502
-
-
Thai, L.J.1
-
37
-
-
9544255791
-
A controlled trial of idebenone in Huntington's disease
-
Ranen NG et al. (1996) A controlled trial of idebenone in Huntington's disease. Mov Disord 11: 549-554
-
(1996)
Mov Disord
, vol.11
, pp. 549-554
-
-
Ranen, N.G.1
-
38
-
-
0033533071
-
Effect at idebenone on cardiomyopathy in Friedreich's ataxia: A preliminary study
-
Rustin P et al. (1999) Effect at idebenone on cardiomyopathy in Friedreich's ataxia: A preliminary study. Lancet 354: 477-479
-
(1999)
Lancet
, vol.354
, pp. 477-479
-
-
Rustin, P.1
-
39
-
-
0037849955
-
Idebenone treatment in Friedreich patients: One-year-long randomized placebo-controlled trial
-
Mariotti C et al. (2003) Idebenone treatment in Friedreich patients: One-year-long randomized placebo-controlled trial. Neurology 60: 1676-1679
-
(2003)
Neurology
, vol.60
, pp. 1676-1679
-
-
Mariotti, C.1
-
40
-
-
34247159966
-
Neurological, cardiological, and oculomotor progression in 104 patients with Friedreich ataxia during long-term follow-up
-
Ribai P et al. (2007) Neurological, cardiological, and oculomotor progression in 104 patients with Friedreich ataxia during long-term follow-up. Arch Neurol 64: 558-664.
-
(2007)
Arch Neurol
, vol.64
, pp. 558-664
-
-
Ribai, P.1
-
41
-
-
2542556601
-
Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia
-
Seznec H et al. (2004) Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia. Hum Mol Genet 13:1017-1024
-
(2004)
Hum Mol Genet
, vol.13
, pp. 1017-1024
-
-
Seznec, H.1
-
42
-
-
2442495100
-
Cerebrospinal fluid concentrations of idebenone in Friedleich ataxia patients
-
Artuch R et al. (2004) Cerebrospinal fluid concentrations of idebenone in Friedleich ataxia patients. Neuropediatrics 35: 95-98
-
(2004)
Neuropediatrics
, vol.35
, pp. 95-98
-
-
Artuch, R.1
-
43
-
-
34250338864
-
Safety, tolerability, and pharmacokinetics of high-dose idebenone administered to patients with Friedreich's ataxia
-
Di Prospero NA et al. (2007) Safety, tolerability, and pharmacokinetics of high-dose idebenone administered to patients with Friedreich's ataxia. Arch Neurol 64: 803-808
-
(2007)
Arch Neurol
, vol.64
, pp. 803-808
-
-
Di Prospero, N.A.1
-
44
-
-
34548606803
-
Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: A randomised placebo-controlled trial
-
Di Prospero N et al. (2007) Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: A randomised placebo-controlled trial. Lancet Neurol 6: 878-886
-
(2007)
Lancet Neurol
, vol.6
, pp. 878-886
-
-
Di Prospero, N.1
-
45
-
-
34250664327
-
MitoQ-a mitochondria-targeted antioxidant
-
Tauskela JS (2007) MitoQ-a mitochondria-targeted antioxidant. IDrugs 10: 399-412
-
(2007)
IDrugs
, vol.10
, pp. 399-412
-
-
Tauskela, J.S.1
-
46
-
-
37649021876
-
Friedreich's ataxia and related loss-of-function disorders
-
2W7, Ed, San Diego: Academic Press
-
Pandolfo M and Dupondt C (2W7) Friedreich's ataxia and related loss-of-function disorders. In Molecular Neurology, 277-294 (Ed Waxman SG) San Diego: Academic Press
-
Molecular Neurology
, pp. 277-294
-
-
Pandolfo, M.1
Dupondt, C.2
-
47
-
-
33847645440
-
DNA single-strand break repair is impaired in aprataxin-related ataxia
-
Hirano M et al. (2007) DNA single-strand break repair is impaired in aprataxin-related ataxia. Ann Neurol 61: 162-174.
-
(2007)
Ann Neurol
, vol.61
, pp. 162-174
-
-
Hirano, M.1
-
48
-
-
34250775522
-
Senataxin, defective in ataxia oculomotor apraxia type 2 is involved in the defense against oxidative DNA damage
-
Suraweera A et al. (2007) Senataxin, defective in ataxia oculomotor apraxia type 2 is involved in the defense against oxidative DNA damage. J Cell Biol 177: 969-979
-
(2007)
J Cell Biol
, vol.177
, pp. 969-979
-
-
Suraweera, A.1
-
49
-
-
26444513273
-
Impaired genomic stability and increased oxidative stress exacerbate different features of ataxia-telangiectasia
-
Ziv S et al. (2005) Impaired genomic stability and increased oxidative stress exacerbate different features of ataxia-telangiectasia. Hum Mol Genet 14: 2929-2943
-
(2005)
Hum Mol Genet
, vol.14
, pp. 2929-2943
-
-
Ziv, S.1
-
50
-
-
33747371969
-
Dramatic extension of tumor latency and correction of neurobehavioral phenotype in Atm-mutant mice with a nitroxide antioxidant
-
Gueven M et al. (2006) Dramatic extension of tumor latency and correction of neurobehavioral phenotype in Atm-mutant mice with a nitroxide antioxidant. Free Radic Biol Med 41: 992-1000
-
(2006)
Free Radic Biol Med
, vol.41
, pp. 992-1000
-
-
Gueven, M.1
-
51
-
-
33746079988
-
Effect of the reduction of superoxide dismutase 1 and 2 or treatment with alpha-tocopherol on tumorigenesis in Atm-deficient mice
-
Erker L et al. (2006) Effect of the reduction of superoxide dismutase 1 and 2 or treatment with alpha-tocopherol on tumorigenesis in Atm-deficient mice. Free Radic Biol Med 41: 590-600
-
(2006)
Free Radic Biol Med
, vol.41
, pp. 590-600
-
-
Erker, L.1
-
52
-
-
34447333096
-
Spinocerebellar ataxias: An update
-
Soong BW and Paulson HL (2007) Spinocerebellar ataxias: An update. Curr Opin Neurol 20:438-446
-
(2007)
Curr Opin Neurol
, vol.20
, pp. 438-446
-
-
Soong, B.W.1
Paulson, H.L.2
-
53
-
-
0042126676
-
Experimental therapeutics in Huntington's disease: Are models useful for therapeutic trials?
-
Bates GP and Hockly E (2003) Experimental therapeutics in Huntington's disease: Are models useful for therapeutic trials? Curr Opin Neurol 16: 465-470
-
(2003)
Curr Opin Neurol
, vol.16
, pp. 465-470
-
-
Bates, G.P.1
Hockly, E.2
-
54
-
-
33744526447
-
Cerebellar ataxia with coenzyme Q10 deficiency: Diagnosis and follow-up after coenzyme Q10 supplementation
-
Artuch R et al. (2006) Cerebellar ataxia with coenzyme Q10 deficiency: Diagnosis and follow-up after coenzyme Q10 supplementation. J Neurol Sci 246: 163-158
-
(2006)
J Neurol Sci
, vol.246
, pp. 163-158
-
-
Artuch, R.1
-
55
-
-
1942453308
-
The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants
-
Mattiazzi M et al. (2004) The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants. Hum Mol Genet 13 869-879
-
(2004)
Hum Mol Genet
, vol.13
, pp. 869-879
-
-
Mattiazzi, M.1
-
56
-
-
0034642203
-
Oxidative stress in patients with Friedreich's ataxia
-
Schulz JB et al. (2000) Oxidative stress in patients with Friedreich's ataxia. Neurology 55: 1719-1721
-
(2000)
Neurology
, vol.55
, pp. 1719-1721
-
-
Schulz, J.B.1
-
57
-
-
0036694802
-
Friedreich's ataxia: Idebenone treatment in early stage patients
-
Artuch R et al. (2002) Friedreich's ataxia: Idebenone treatment in early stage patients. Neuropediatrics 33: 130-193
-
(2002)
Neuropediatrics
, vol.33
, pp. 130-193
-
-
Artuch, R.1
-
58
-
-
0036221156
-
Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia
-
Hausse AO et al. (2002) Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia. Heart 87: 346-349
-
(2002)
Heart
, vol.87
, pp. 346-349
-
-
Hausse, A.O.1
-
59
-
-
0036554660
-
Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia
-
Rustin P et al. (2002) Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia. Free Radic Res 36: 467-469
-
(2002)
Free Radic Res
, vol.36
, pp. 467-469
-
-
Rustin, P.1
-
60
-
-
0038187688
-
Idebenone treatment in Friedreich's ataxia: Neurological, cardiac, and biochemical monitoring
-
Buyse G et al. (2003) Idebenone treatment in Friedreich's ataxia: neurological, cardiac, and biochemical monitoring. Neurology 60 1679-1681
-
(2003)
Neurology
, vol.60
, pp. 1679-1681
-
-
Buyse, G.1
|