-
1
-
-
0037101845
-
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., and Lill R. The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins. Hum. Mol. Genet. 11 (2002) 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
-
2
-
-
0037613459
-
Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins
-
Yoon T., and Cowan J.A. Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins. J. Am. Chem. Soc. 125 (2003) 6078-6084
-
(2003)
J. Am. Chem. Soc.
, vol.125
, pp. 6078-6084
-
-
Yoon, T.1
Cowan, J.A.2
-
3
-
-
0041808717
-
Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells
-
Tan G., Napoli E., Taroni F., and Cortopassi G.A. Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells. Hum. Mol. Genet. 12 (2003) 1699-1711
-
(2003)
Hum. Mol. Genet.
, vol.12
, pp. 1699-1711
-
-
Tan, G.1
Napoli, E.2
Taroni, F.3
Cortopassi, G.A.4
-
4
-
-
29644442275
-
Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells
-
Schoenfeld R.A., Napoli E., Wong A., Zhan S., Reutenauer L., Morin D., Buckpitt R., Taroni F., Lonnerdal B., Ristow M., Puccio H., and Cortopassi G.A. Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells. Hum. Mol. Genet. 14 (2005) 3787-3799
-
(2005)
Hum. Mol. Genet.
, vol.14
, pp. 3787-3799
-
-
Schoenfeld, R.A.1
Napoli, E.2
Wong, A.3
Zhan, S.4
Reutenauer, L.5
Morin, D.6
Buckpitt, R.7
Taroni, F.8
Lonnerdal, B.9
Ristow, M.10
Puccio, H.11
Cortopassi, G.A.12
-
5
-
-
33646696625
-
Frataxin, iron-sulfur clusters, heme, ROS, and aging
-
Napoli E., Taroni F., and Cortopassi G.A. Frataxin, iron-sulfur clusters, heme, ROS, and aging. Antioxid. Redox Signal. 8 (2006) 506-516
-
(2006)
Antioxid. Redox Signal.
, vol.8
, pp. 506-516
-
-
Napoli, E.1
Taroni, F.2
Cortopassi, G.A.3
-
6
-
-
33845693952
-
Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts
-
Lu C., and Cortopassi G.A. Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts. Arch. Biochem. Biophys. 457 (2007) 111-122
-
(2007)
Arch. Biochem. Biophys.
, vol.457
, pp. 111-122
-
-
Lu, C.1
Cortopassi, G.A.2
-
7
-
-
3142722152
-
The heme synthesis defect of mutants impaired in mitochondrial iron-sulfur protein biogenesis is caused by reversible inhibition of ferrochelatase
-
Lange H., Muhlenhoff U., Denzel M., Kispal G., and Lill R. The heme synthesis defect of mutants impaired in mitochondrial iron-sulfur protein biogenesis is caused by reversible inhibition of ferrochelatase. J. Biol. Chem. 279 (2004) 29101-29108
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 29101-29108
-
-
Lange, H.1
Muhlenhoff, U.2
Denzel, M.3
Kispal, G.4
Lill, R.5
-
8
-
-
4644221775
-
Iron-sulfur cluster biosynthesis: toward an understanding of cellular machinery and molecular mechanism
-
Mansy S.S., and Cowan J.A. Iron-sulfur cluster biosynthesis: toward an understanding of cellular machinery and molecular mechanism. Acc. Chem. Res. 37 (2004) 719-725
-
(2004)
Acc. Chem. Res.
, vol.37
, pp. 719-725
-
-
Mansy, S.S.1
Cowan, J.A.2
-
9
-
-
33644623262
-
Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis
-
Tong H., and Rouault T.A. Functions of mitochondrial ISCU and cytosolic ISCU in mammalian iron-sulfur cluster biogenesis and iron homeostasis. Cell Metab. 3 (2006) 199-210
-
(2006)
Cell Metab.
, vol.3
, pp. 199-210
-
-
Tong, H.1
Rouault, T.A.2
-
10
-
-
0037155866
-
Mitochondrial ferredoxin is required for heme A synthesis in Saccharomyces cerevisiae
-
Barros M.H., Nobrega F.G., and Tzagoloff A. Mitochondrial ferredoxin is required for heme A synthesis in Saccharomyces cerevisiae. J. Biol. Chem. 277 (2002) 9997-10002
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 9997-10002
-
-
Barros, M.H.1
Nobrega, F.G.2
Tzagoloff, A.3
-
11
-
-
14844362079
-
Recommendations for the diagnosis and treatment of the acute porphyrias
-
Anderson K.E., Bloomer J.R., Bonkovsky H.L., Kushner J.P., Pierach C.A., Pimstone N.R., and Desnick R.J. Recommendations for the diagnosis and treatment of the acute porphyrias. Ann. Intern. Med. 142 (2005) 439-450
-
(2005)
Ann. Intern. Med.
, vol.142
, pp. 439-450
-
-
Anderson, K.E.1
Bloomer, J.R.2
Bonkovsky, H.L.3
Kushner, J.P.4
Pierach, C.A.5
Pimstone, N.R.6
Desnick, R.J.7
-
12
-
-
0038448961
-
Upregulation of expression from the FRDA genomic locus for the therapy of Friedreich ataxia
-
Sarsero J.P., Li L., Wardan H., Sitte K., Williamson R., and Ioannou P.A. Upregulation of expression from the FRDA genomic locus for the therapy of Friedreich ataxia. J. Gene Med. 5 (2003) 72-81
-
(2003)
J. Gene Med.
, vol.5
, pp. 72-81
-
-
Sarsero, J.P.1
Li, L.2
Wardan, H.3
Sitte, K.4
Williamson, R.5
Ioannou, P.A.6
-
13
-
-
0036799372
-
Non-essential function for yeast frataxin in iron-sulfur cluster assembly
-
Duby G., Foury F., Ramazzotti A., Herrmann J., and Lutz T. Non-essential function for yeast frataxin in iron-sulfur cluster assembly. Hum. Mol. Genet. 11 (2002) 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
-
-
0037447390
-
Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1)
-
Lesuisse E., Santos R., Matzanke B.F., Knight S.A., Camadro J.M., and Dancis A. Iron use for haeme synthesis is under control of the yeast frataxin homologue (Yfh1). Hum. Mol. Genet. 12 (2003) 879-889
-
(2003)
Hum. Mol. Genet.
, vol.12
, pp. 879-889
-
-
Lesuisse, E.1
Santos, R.2
Matzanke, B.F.3
Knight, S.A.4
Camadro, J.M.5
Dancis, A.6
-
15
-
-
0042232045
-
Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation
-
Park S., Gakh O., O'Neill H.A., Mangravita A., Nichol H., Ferreira G.C., and Isaya G. Yeast frataxin sequentially chaperones and stores iron by coupling protein assembly with iron oxidation. J. Biol. Chem. 278 (2003) 31340-31351
-
(2003)
J. Biol. Chem.
, vol.278
, pp. 31340-31351
-
-
Park, S.1
Gakh, O.2
O'Neill, H.A.3
Mangravita, A.4
Nichol, H.5
Ferreira, G.C.6
Isaya, G.7
-
16
-
-
2942744572
-
Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis
-
Yoon T., and Cowan J.A. Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis. J. Biol. Chem. 279 (2004) 25943-25946
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 25943-25946
-
-
Yoon, T.1
Cowan, J.A.2
-
17
-
-
33748154507
-
Mitochondrial disease activates transcripts of the unfolded protein response and cell cycle and inhibits vesicular secretion and oligodendrocyte-specific transcripts
-
Cortopassi G.A., Danielson S., Alemi M., Zhan S.S., Tong W., Carelli V., Martinuzzi A., Marzuky S., Majamaa K., and Wong A. Mitochondrial disease activates transcripts of the unfolded protein response and cell cycle and inhibits vesicular secretion and oligodendrocyte-specific transcripts. Mitochondrion 6 (2006) 161-175
-
(2006)
Mitochondrion
, vol.6
, pp. 161-175
-
-
Cortopassi, G.A.1
Danielson, S.2
Alemi, M.3
Zhan, S.S.4
Tong, W.5
Carelli, V.6
Martinuzzi, A.7
Marzuky, S.8
Majamaa, K.9
Wong, A.10
-
18
-
-
0037051889
-
Regulation of the heme A biosynthetic pathway in Saccharomyces cerevisiae
-
Barros M.H., and Tzagoloff A. Regulation of the heme A biosynthetic pathway in Saccharomyces cerevisiae. FEBS Lett. 516 (2002) 119-123
-
(2002)
FEBS Lett.
, vol.516
, pp. 119-123
-
-
Barros, M.H.1
Tzagoloff, A.2
-
19
-
-
0037155866
-
Mitochondrial ferredoxin is required for heme A synthesis in Saccharomyces cerevisiae
-
Barros M.H., Nobrega F.G., and Tzagoloff A. Mitochondrial ferredoxin is required for heme A synthesis in Saccharomyces cerevisiae. J. Biol. Chem. 277 (2002) 9997-10002
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 9997-10002
-
-
Barros, M.H.1
Nobrega, F.G.2
Tzagoloff, A.3
-
20
-
-
0034636795
-
IscU as a scaffold for iron-sulfur cluster biosynthesis: sequential assembly of [2Fe-2S] and [4Fe-4S] clusters in IscU
-
Agar J.N., Krebs C., Frazzon J., Huynh B.H., Dean D.R., and Johnson M.K. IscU as a scaffold for iron-sulfur cluster biosynthesis: sequential assembly of [2Fe-2S] and [4Fe-4S] clusters in IscU. Biochemistry 39 (2000) 7856-7862
-
(2000)
Biochemistry
, vol.39
, pp. 7856-7862
-
-
Agar, J.N.1
Krebs, C.2
Frazzon, J.3
Huynh, B.H.4
Dean, D.R.5
Johnson, M.K.6
-
21
-
-
3042613609
-
Heme, iron and the mitochondrial decay of ageing
-
Atamna H. Heme, iron and the mitochondrial decay of ageing. Ageing Res. Rev. 3 (2004) 303-318
-
(2004)
Ageing Res. Rev.
, vol.3
, pp. 303-318
-
-
Atamna, H.1
-
22
-
-
0021137263
-
Porphyrin-heme biosynthesis in organotypic cultures of mouse dorsal root ganglia. Effects of heme and lead on porphyrin synthesis and peripheral myelin
-
Whetsell W.O., Sassa S., and Kappas A. Porphyrin-heme biosynthesis in organotypic cultures of mouse dorsal root ganglia. Effects of heme and lead on porphyrin synthesis and peripheral myelin. J. Clin. Invest. 74 (1984) 600-607
-
(1984)
J. Clin. Invest.
, vol.74
, pp. 600-607
-
-
Whetsell, W.O.1
Sassa, S.2
Kappas, A.3
-
23
-
-
78651050757
-
The process of demyelination in the central nervous system: IV. Demyelination as a delayed residual of carbon monoxide asphyxia
-
Courville C.B. The process of demyelination in the central nervous system: IV. Demyelination as a delayed residual of carbon monoxide asphyxia. J. Nerv. Ment. Dis. 125 (1957) 534-546
-
(1957)
J. Nerv. Ment. Dis.
, vol.125
, pp. 534-546
-
-
Courville, C.B.1
-
24
-
-
0021018858
-
Cyanide in human disease: a review of clinical and laboratory evidence
-
Wilson J. Cyanide in human disease: a review of clinical and laboratory evidence. Fundam. Appl. Toxicol. 3 (1983) 397-399
-
(1983)
Fundam. Appl. Toxicol.
, vol.3
, pp. 397-399
-
-
Wilson, J.1
-
25
-
-
0035198691
-
The neurotoxicant, Cuprizone, as a model to study demyelination and remyelination in the central nervous system
-
Matsushima G.K., and Morell P. The neurotoxicant, Cuprizone, as a model to study demyelination and remyelination in the central nervous system. Brain Pathol. 11 (2001) 107
-
(2001)
Brain Pathol.
, vol.11
, pp. 107
-
-
Matsushima, G.K.1
Morell, P.2
-
26
-
-
0033554327
-
A family with Friedreich's ataxia and onion-bulb formations at sural nerve biopsy
-
Barreira A.A., Marques W., Sweeney M.G., Davis M.B., Chimelli L., Paçó-Larson M.L., and Wood N.W. A family with Friedreich's ataxia and onion-bulb formations at sural nerve biopsy. Ann. N.Y. Acad. Sci. 883 (1999) 466
-
(1999)
Ann. N.Y. Acad. Sci.
, vol.883
, pp. 466
-
-
Barreira, A.A.1
Marques, W.2
Sweeney, M.G.3
Davis, M.B.4
Chimelli, L.5
Paçó-Larson, M.L.6
Wood, N.W.7
-
27
-
-
33749638768
-
GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology
-
Al-Mahdawi S., Mouro Pinto R., Varshney D., Lawrence L., Lowrie M.B., Hughes S., Webster Z., Blake J., Cooper J.M., King R., and Pook M.A. GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology. Genomics 88 (2006) 580-590
-
(2006)
Genomics
, vol.88
, pp. 580-590
-
-
Al-Mahdawi, S.1
Mouro Pinto, R.2
Varshney, D.3
Lawrence, L.4
Lowrie, M.B.5
Hughes, S.6
Webster, Z.7
Blake, J.8
Cooper, J.M.9
King, R.10
Pook, M.A.11
-
28
-
-
0032488980
-
Iron differentially stimulates translation of mitochondrial aconitase and ferritin mRNAs in mammalian cells
-
Schalinske K.L., Chen O.S., and Eisenstein R.S. Iron differentially stimulates translation of mitochondrial aconitase and ferritin mRNAs in mammalian cells. J. Biol. Chem. 273 (1998) 3740-3746
-
(1998)
J. Biol. Chem.
, vol.273
, pp. 3740-3746
-
-
Schalinske, K.L.1
Chen, O.S.2
Eisenstein, R.S.3
-
29
-
-
27844526573
-
Recombinant human erythropoietin: effects on frataxin expression in vitro
-
Sturm B., Stupphann D., Kaun C., Boesch S., Schranzhofer M., Wojta J., Goldenberg H., and Scheiber-Mojdehkar B. Recombinant human erythropoietin: effects on frataxin expression in vitro. Eur. J. Clin. Invest. 35 (2005) 711-717
-
(2005)
Eur. J. Clin. Invest.
, vol.35
, pp. 711-717
-
-
Sturm, B.1
Stupphann, D.2
Kaun, C.3
Boesch, S.4
Schranzhofer, M.5
Wojta, J.6
Goldenberg, H.7
Scheiber-Mojdehkar, B.8
-
30
-
-
0033533071
-
Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study
-
Rustin P., von Kleist-Retzow J.C., Chantrel-Groussard K., Sidi D., Munnich A., and Rotig A. Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study. Lancet 354 (1999) 477-479
-
(1999)
Lancet
, vol.354
, pp. 477-479
-
-
Rustin, P.1
von Kleist-Retzow, J.C.2
Chantrel-Groussard, K.3
Sidi, D.4
Munnich, A.5
Rotig, A.6
-
31
-
-
0036221156
-
Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia
-
Hausse A.O., Aggoun Y., Bonnet D., Sidi D., Munnich A., Rotig A., and Rustin P. Idebenone 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
Rustin, P.7
-
32
-
-
0036554660
-
Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia
-
Rustin P., Rotig A., Munnich A., and Sidi D. Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia. Free Radical Res. 36 (2002) 467-469
-
(2002)
Free Radical Res.
, vol.36
, pp. 467-469
-
-
Rustin, P.1
Rotig, A.2
Munnich, A.3
Sidi, D.4
-
33
-
-
0036799799
-
Antioxidants as treatment for neurodegenerative disorders
-
Moosmann B., and Behl C. Antioxidants as treatment for neurodegenerative disorders. Expert Opin. Investig. Drugs 11 (2002) 1407-1435
-
(2002)
Expert Opin. Investig. Drugs
, vol.11
, pp. 1407-1435
-
-
Moosmann, B.1
Behl, C.2
-
34
-
-
0348136710
-
Mitochondria-targeted antioxidants protect Friedreich Ataxia fibroblasts from endogenous oxidative stress more effectively than untargeted antioxidants
-
Jauslin M.L., Meier T., Smith R.A., and Murphy M.P. Mitochondria-targeted antioxidants protect Friedreich Ataxia fibroblasts from endogenous oxidative stress more effectively than untargeted antioxidants. FASEB J. 17 (2003) 1972-1974
-
(2003)
FASEB J.
, vol.17
, pp. 1972-1974
-
-
Jauslin, M.L.1
Meier, T.2
Smith, R.A.3
Murphy, M.P.4
-
35
-
-
18344366874
-
Role of mitochondria in oxidative stress and aging
-
Lenaz G., Bovina C., D'Aurelio M., Fato R., Formiggini G., Genova M.L., Giuliano G., Merlo Pich M., Paolucci U., Parenti Castelli G., and Ventura B. Role of mitochondria in oxidative stress and aging. Ann. N.Y. Acad. Sci. 959 (2002) 199-213
-
(2002)
Ann. N.Y. Acad. Sci.
, vol.959
, pp. 199-213
-
-
Lenaz, G.1
Bovina, C.2
D'Aurelio, M.3
Fato, R.4
Formiggini, G.5
Genova, M.L.6
Giuliano, G.7
Merlo Pich, M.8
Paolucci, U.9
Parenti Castelli, G.10
Ventura, B.11
-
36
-
-
0025748654
-
Intravenous hemealbumin in acute intermittent porphyria: evidence for repletion of hepatic hemoproteins and regulatory heme pools
-
Bonkovsky H.L., Healey J.F., Lourie A.N., and Gerron G.G. Intravenous hemealbumin in acute intermittent porphyria: evidence for repletion of hepatic hemoproteins and regulatory heme pools. Am. J. Gastroenterol. 86 (1991) 1050-1056
-
(1991)
Am. J. Gastroenterol.
, vol.86
, pp. 1050-1056
-
-
Bonkovsky, H.L.1
Healey, J.F.2
Lourie, A.N.3
Gerron, G.G.4
-
37
-
-
0033771110
-
New insights into the role of CcmC, CcmD and CcmE in the haem delivery pathway during cytochrome c maturation by a complete mutational analysis of the conserved tryptophan-rich motif of CcmC
-
Schulz H., Pellicioli E.C., and Thony-Meyer L. New insights into the role of CcmC, CcmD and CcmE in the haem delivery pathway during cytochrome c maturation by a complete mutational analysis of the conserved tryptophan-rich motif of CcmC. Mol. Microbiol. 37 (2000) 1379-1388
-
(2000)
Mol. Microbiol.
, vol.37
, pp. 1379-1388
-
-
Schulz, H.1
Pellicioli, E.C.2
Thony-Meyer, L.3
-
38
-
-
0034778511
-
Frataxin expression rescues mitochondrial dysfunctions in FRDA cells
-
Tan G., Chen L.S., Lonnerdal B., Gellera C., Taroni F.A., and Cortopassi G.A. Frataxin expression rescues mitochondrial dysfunctions in FRDA cells. Hum. Mol. Genet. 10 (2001) 2099-2107
-
(2001)
Hum. Mol. Genet.
, vol.10
, pp. 2099-2107
-
-
Tan, G.1
Chen, L.S.2
Lonnerdal, B.3
Gellera, C.4
Taroni, F.A.5
Cortopassi, G.A.6
-
39
-
-
0032513350
-
Cytochrome oxidase assay in minute amounts of skeletal muscle using single wavelength spectrophotometers
-
Miró O., Cardellach F., Barrientos A., Casademont J., Rötig A., and Rustin P. Cytochrome oxidase assay in minute amounts of skeletal muscle using single wavelength spectrophotometers. J. Neurosci. Methods 80 (1998) 107-111
-
(1998)
J. Neurosci. Methods
, vol.80
, pp. 107-111
-
-
Miró, O.1
Cardellach, F.2
Barrientos, A.3
Casademont, J.4
Rötig, A.5
Rustin, P.6
-
40
-
-
0027979002
-
C-terminal region of Adrenodoxin affects its structural integrity and determines differences in its electron transfer function to cytochrome P-450
-
Uhlmann H., Kraft R., and Bernhardt R. C-terminal region of Adrenodoxin affects its structural integrity and determines differences in its electron transfer function to cytochrome P-450. J. Biol. Chem. 269 (1994) 22557-22564
-
(1994)
J. Biol. Chem.
, vol.269
, pp. 22557-22564
-
-
Uhlmann, H.1
Kraft, R.2
Bernhardt, R.3
|