-
1
-
-
77953902544
-
Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches
-
Schmucker S, Puccio H. Understanding the molecular mechanisms of Friedreich's ataxia to develop therapeutic approaches. Hum Mol Genet 2010; 19: R103-R110.
-
(2010)
Hum Mol Genet
, vol.19
-
-
Schmucker, S.1
Puccio, H.2
-
2
-
-
0034192352
-
Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation
-
Cossee M, Puccio H, Gansmuller A, Koutnikova H, Dierich A, LeMeur M et al. Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation. Hum Mol Genet 2000; 9: 1219-1226. (Pubitemid 30248608)
-
(2000)
Human Molecular Genetics
, vol.9
, Issue.8
, pp. 1219-1226
-
-
Cossee, M.1
Puccio, H.2
Gansmuller, A.3
Koutnikova, H.4
Dierich, A.5
LeMeur, M.6
Fischbeck, K.7
Dolle, P.8
Koenig, M.9
-
3
-
-
33846782968
-
Knockout of frataxin gene causes embryo lethality in Arabidopsis
-
DOI 10.1016/j.febslet.2007.01.030, PII S0014579307000580
-
Vazzola V, Losa A, Soave C, Murgia I. Knockout of frataxin gene causes embryo lethality in Arabidopsis. FEBS Lett 2007; 581: 667-672. (Pubitemid 46216291)
-
(2007)
FEBS Letters
, vol.581
, Issue.4
, pp. 667-672
-
-
Vazzola, V.1
Losa, A.2
Soave, C.3
Murgia, I.4
-
4
-
-
22144462536
-
Reduced expression of frataxin extends the lifespan of Caenorhabditis elegans
-
DOI 10.1111/j.1474-9726.2005.00149.x
-
Ventura N, Rea S, Henderson ST, Condo I, Johnson TE, Testi R. Reduced expression of frataxin extends the lifespan of Caenorhabditis elegans. Aging Cell 2005; 4: 109-112. (Pubitemid 41632910)
-
(2005)
Aging Cell
, vol.4
, Issue.2
, pp. 109-112
-
-
Ventura, N.1
Rea, S.2
Henderson, S.T.3
Condo, I.4
Johnson, T.E.5
Testi, R.6
-
5
-
-
77951253200
-
The ins and outs of mitochondrial iron-loading: The metabolic defect in Friedreich's ataxia
-
Richardson DR, Huang ML, Whitnall M, Becker EM, Ponka P, Rahmanto YS. The ins and outs of mitochondrial iron-loading: the metabolic defect in Friedreich's ataxia. J Mol Med 2010; 88: 323-329.
-
(2010)
J Mol Med
, vol.88
, pp. 323-329
-
-
Richardson, D.R.1
Huang, M.L.2
Whitnall, M.3
Becker, E.M.4
Ponka, P.5
Rahmanto, Y.S.6
-
7
-
-
33745222541
-
A pool of extramitochondrial frataxin that promotes cell survival
-
Condo I, Ventura N, Malisan F, Tomassini B, Testi R. A pool of extramitochondrial frataxin that promotes cell survival. J Biol Chem 2006; 281: 16750-16756.
-
(2006)
J Biol Chem
, vol.281
, pp. 16750-16756
-
-
Condo, I.1
Ventura, N.2
Malisan, F.3
Tomassini, B.4
Testi, R.5
-
8
-
-
9744248303
-
Iron-sulfur protein maturation in human cells: Evidence for a function of frataxin
-
DOI 10.1093/hmg/ddh324
-
Stehling O, Elsasser HP, Bruckel B, Muhlenhoff U, Lill R. Iron-sulfur protein maturation in human cells: evidence for a function of frataxin. Hum Mol Genet 2004; 13: 3007-3015. (Pubitemid 39585233)
-
(2004)
Human Molecular Genetics
, vol.13
, Issue.23
, pp. 3007-3015
-
-
Stehling, O.1
Elsasser, H.2
Bruckel, B.3
Muhlenhoff, U.4
Lill, R.5
-
9
-
-
29644448054
-
Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice
-
DOI 10.1093/hmg/ddi410
-
Thierbach R, Schulz TJ, Isken F, Voigt A, Mietzner B, Drewes G et al. Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice. Hum Mol Genet 2005; 14: 3857-3864. (Pubitemid 43020087)
-
(2005)
Human Molecular Genetics
, vol.14
, Issue.24
, pp. 3857-3864
-
-
Thierbach, R.1
Schulz, T.J.2
Isken, F.3
Voigt, A.4
Mietzner, B.5
Drewes, G.6
Von Kleist-Retzow, J.-C.7
Wiesner, R.J.8
Magnuson, M.A.9
Puccio, H.10
Pfeiffer, A.F.H.11
Steinberg, P.12
Ristow, M.13
-
10
-
-
70349575790
-
Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screen
-
Calmels N, Seznec H, Villa P, Reutenauer L, Hibert M, Haiech J et al. Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screen. BMC Neurol 2009; 9: 46.
-
(2009)
BMC Neurol
, vol.9
, pp. 46
-
-
Calmels, N.1
Seznec, H.2
Villa, P.3
Reutenauer, L.4
Hibert, M.5
Haiech, J.6
-
11
-
-
33644868755
-
Induction of oxidative metabolism by mitochondrial frataxin inhibits cancer growth: Otto Warburg revisited
-
Schulz TJ, Thierbach R, Voigt A, Drewes G, Mietzner B, Steinberg P et al. Induction of oxidative metabolism by mitochondrial frataxin inhibits cancer growth: Otto Warburg revisited. J Biol Chem 2006; 281: 977-981.
-
(2006)
J Biol Chem
, vol.281
, pp. 977-981
-
-
Schulz, T.J.1
Thierbach, R.2
Voigt, A.3
Drewes, G.4
Mietzner, B.5
Steinberg, P.6
-
12
-
-
34748841015
-
Small molecules affecting transcription in Friedreich ataxia
-
DOI 10.1016/j.pharmthera.2007.06.014, PII S0163725807001519
-
Gottesfeld JM. Small molecules affecting transcription in Friedreich ataxia. Pharmacol Ther 2007; 116: 236-248. (Pubitemid 47469990)
-
(2007)
Pharmacology and Therapeutics
, vol.116
, Issue.2
, pp. 236-248
-
-
Gottesfeld, J.M.1
-
13
-
-
67949124861
-
PPAR-gamma agonist Azelaoyl PAF increases frataxin protein and mRNA expression: New implications for the Friedreich's ataxia therapy
-
Marmolino D, Acquaviva F, Pinelli M, Monticelli A, Castaldo I, Filla A et al. PPAR-gamma agonist Azelaoyl PAF increases frataxin protein and mRNA expression: new implications for the Friedreich's ataxia therapy. Cerebellum 2009; 8: 98-103.
-
(2009)
Cerebellum
, vol.8
, pp. 98-103
-
-
Marmolino, D.1
Acquaviva, F.2
Pinelli, M.3
Monticelli, A.4
Castaldo, I.5
Filla, A.6
-
14
-
-
34249704041
-
Hypoxia-inducible factor 2α regulates expression of the mitochondrial aconitase chaperone protein frataxin
-
DOI 10.1074/jbc.M611133200
-
Oktay Y, Dioum E, Matsuzaki S, Ding K, Yan LJ, Haller RG et al. Hypoxia-inducible factor 2alpha regulates expression of the mitochondrial aconitase chaperone protein frataxin. J Biol Chem 2007; 282: 11750-11756. (Pubitemid 47100692)
-
(2007)
Journal of Biological Chemistry
, vol.282
, Issue.16
, pp. 11750-11756
-
-
Oktay, Y.1
Dioum, E.2
Matsuzaki, S.3
Ding, K.4
Yan, L.-J.5
Haller, R.G.6
Szweda, L.I.7
Garcia, J.A.8
-
15
-
-
70350244837
-
Hypoxia, angiogenesis and cancer therapy: To breathe or not to breathe?
-
Michieli P. Hypoxia, angiogenesis and cancer therapy: to breathe or not to breathe? Cell Cycle 2009; 8: 3291-3296.
-
(2009)
Cell Cycle
, vol.8
, pp. 3291-3296
-
-
Michieli, P.1
-
16
-
-
76349095132
-
Defining the role of hypoxia-inducible factor 1 in cancer biology and therapeutics
-
Semenza GL. Defining the role of hypoxia-inducible factor 1 in cancer biology and therapeutics. Oncogene 2010; 29: 625-634.
-
(2010)
Oncogene
, vol.29
, pp. 625-634
-
-
Semenza, G.L.1
-
17
-
-
76049100577
-
HIF-1: Upstream and downstream of cancer metabolism
-
Semenza GL. HIF-1: upstream and downstream of cancer metabolism. Curr Opin Genet Dev 2010; 20: 51-56.
-
(2010)
Curr Opin Genet Dev
, vol.20
, pp. 51-56
-
-
Semenza, G.L.1
-
18
-
-
34247477207
-
Hypoxia inducible factor-2alpha in cancer
-
Lofstedt T, Fredlund E, Holmquist-Mengelbier L, Pietras A, Ovenberger M, Poellinger L et al. Hypoxia inducible factor-2alpha in cancer. Cell Cycle 2007; 6: 919-926.
-
(2007)
Cell Cycle
, vol.6
, pp. 919-926
-
-
Lofstedt, T.1
Fredlund, E.2
Holmquist-Mengelbier, L.3
Pietras, A.4
Ovenberger, M.5
Poellinger, L.6
-
19
-
-
0032568448
-
Stabilization of wild-type p53 by hypoxia-inducible factor 1alpha
-
An WG, Kanekal M, Simon MC, Maltepe E, Blagosklonny MV, Neckers LM. Stabilization of wild-type p53 by hypoxia-inducible factor 1alpha. Nature 1998; 392: 405-408.
-
(1998)
Nature
, vol.392
, pp. 405-408
-
-
An, W.G.1
Kanekal, M.2
Simon, M.C.3
Maltepe, E.4
Blagosklonny, M.V.5
Neckers, L.M.6
-
21
-
-
70149106164
-
HIF2alpha inhibition promotes p53 pathway activity, tumor cell death, and radiation responses
-
Bertout JA, Majmundar AJ, Gordan JD, Lam JC, Ditsworth D, Keith B et al. HIF2alpha inhibition promotes p53 pathway activity, tumor cell death, and radiation responses. Proc Natl Acad Sci USA 2009; 106: 14391-14396.
-
(2009)
Proc Natl Acad Sci USA
, vol.106
, pp. 14391-14396
-
-
Bertout, J.A.1
Majmundar, A.J.2
Gordan, J.D.3
Lam, J.C.4
Ditsworth, D.5
Keith, B.6
-
22
-
-
70349459599
-
Ryan KM. p53 and metabolism
-
Vousden KH, Ryan KM. p53 and metabolism. Nat Rev Cancer 2009; 9: 691-700.
-
(2009)
Nat Rev Cancer
, vol.9
, pp. 691-700
-
-
Vousden, K.H.1
-
24
-
-
20444427605
-
Stress-induced activation of the p53 tumor suppressor in leukemia cells and normal lymphocytes requires mitochondrial activity and reactive oxygen species
-
DOI 10.1182/blood-2004-09-3428
-
Karawajew L, Rhein P, Czerwony G, Ludwig WD. Stress-induced activation of the p53 tumor suppressor in leukemia cells and normal lymphocytes requires mitochondrial activity and reactive oxygen species. Blood 2005; 105: 4767-4775. (Pubitemid 40807302)
-
(2005)
Blood
, vol.105
, Issue.12
, pp. 4767-4775
-
-
Karawajew, L.1
Rhein, P.2
Czerwony, G.3
Ludwig, W.-D.4
-
25
-
-
77954447250
-
Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question
-
Armstrong JS, Khdour O, Hecht SM. Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question. Faseb J 2010; 24: 2152-2163.
-
(2010)
Faseb J
, vol.24
, pp. 2152-2163
-
-
Armstrong, J.S.1
Khdour, O.2
Hecht, S.M.3
-
26
-
-
67651174517
-
Johnson TE. p53/CEP-1 increases or decreases lifespan, depending on level of mitochondrial bioenergetic stress
-
Ventura N, Rea SL, Schiavi A, Torgovnick A, Testi R, Johnson TE. p53/CEP-1 increases or decreases lifespan, depending on level of mitochondrial bioenergetic stress. Aging Cell 2009; 8: 380-393.
-
(2009)
Aging Cell
, vol.8
, pp. 380-393
-
-
Ventura, N.1
Rea, S.L.2
Schiavi, A.3
Torgovnick, A.4
Testi, R.5
-
27
-
-
77953356853
-
A role for p53 in mitochondrial stress response control of longevity in C. elegans
-
Torgovnick A, Schiavi A, Testi R, Ventura N. A role for p53 in mitochondrial stress response control of longevity in C. elegans. Exp Gerontol 2010; 45: 550-557.
-
(2010)
Exp Gerontol
, vol.45
, pp. 550-557
-
-
Torgovnick, A.1
Schiavi, A.2
Testi, R.3
Ventura, N.4
-
28
-
-
64249161639
-
Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia
-
Paupe V, Dassa EP, Goncalves S, Auchere F, Lonn M, Holmgren A et al. Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia. PLoS One 2009; 4: e4253.
-
(2009)
PLoS One
, vol.4
-
-
Paupe, V.1
Dassa, E.P.2
Goncalves, S.3
Auchere, F.4
Lonn, M.5
Holmgren, A.6
-
29
-
-
79952638271
-
Preventing the ubiquitin/proteasome-dependent degradation of frataxin, the protein defective in Friedreich's Ataxia
-
doi: 10.1093/hmg/ddq566
-
Rufini A, Fortuni S, Arcuri G, Condo I, Serio D, Incani O et al. Preventing the ubiquitin/proteasome-dependent degradation of frataxin, the protein defective in Friedreich's Ataxia. Hum Mol Genet 2011, doi: 10.1093/hmg/ddq566.
-
(2011)
Hum Mol Genet
-
-
Rufini, A.1
Fortuni, S.2
Arcuri, G.3
Condo, I.4
Serio, D.5
Incani, O.6
-
30
-
-
77749333034
-
HIF1-positive and HIF1-negative glioblastoma cells compete in vitro but cooperate in tumor growth in vivo
-
Fiorenzo P, Mongiardi MP, Dimitri D, Cozzolino M, Ferri A, Montano N et al. HIF1-positive and HIF1-negative glioblastoma cells compete in vitro but cooperate in tumor growth in vivo. Int J Oncol 2010; 36: 785-791.
-
(2010)
Int J Oncol
, vol.36
, pp. 785-791
-
-
Fiorenzo, P.1
Mongiardi, M.P.2
Dimitri, D.3
Cozzolino, M.4
Ferri, A.5
Montano, N.6
-
31
-
-
0344826529
-
-/- mice
-
DOI 10.1038/ng1266
-
Scortegagna M, Ding K, Oktay Y, Gaur A, Thurmond F, Yan LJ et al. Multiple organ pathology, metabolic abnormalities and impaired homeostasis of reactive oxygen species in Epas1-/- mice. Nat Genet 2003; 35: 331-340. (Pubitemid 37486916)
-
(2003)
Nature Genetics
, vol.35
, Issue.4
, pp. 331-340
-
-
Scortegagna, M.1
Ding, K.2
Oktay, Y.3
Gaur, A.4
Thurmond, F.5
Yan, L.-J.6
Marck, B.T.7
Matsumoto, A.M.8
Shelton, J.M.9
Richardson, J.A.10
Bennet, M.J.11
Garcia, J.A.12
-
32
-
-
71949097180
-
Hypoxia-inducible factor-1alpha blocks differentiation of malignant gliomas
-
Lu H, Li Y, Shu M, Tang J, Huang Y, Zhou Y et al. Hypoxia-inducible factor-1alpha blocks differentiation of malignant gliomas. FEBS J 2009; 276: 7291-7304.
-
(2009)
FEBS J
, vol.276
, pp. 7291-7304
-
-
Lu, H.1
Li, Y.2
Shu, M.3
Tang, J.4
Huang, Y.5
Zhou, Y.6
-
33
-
-
76549110775
-
Frataxin, a molecule of mystery: Trading stability for function in its iron-binding site
-
Lane DJ, Richardson DR. Frataxin, a molecule of mystery: trading stability for function in its iron-binding site. Biochem J 2010; 426: e1-e3.
-
(2010)
Biochem J
, vol.426
-
-
Lane, D.J.1
Richardson, D.R.2
-
34
-
-
70349504414
-
Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant
-
Huang ML, Becker EM, Whitnall M, Rahmanto YS, Ponka P, Richardson DR. Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant. Proc Natl Acad Sci USA 2009; 106: 16381-16386.
-
(2009)
Proc Natl Acad Sci USA
, vol.106
, pp. 16381-16386
-
-
Huang, M.L.1
Becker, E.M.2
Whitnall, M.3
Rahmanto, Y.S.4
Ponka, P.5
Richardson, D.R.6
-
35
-
-
66449124596
-
HIF-2alpha, but not HIF-1alpha, promotes iron absorption in mice
-
Mastrogiannaki M, Matak P, Keith B, Simon MC, Vaulont S, Peyssonnaux C. HIF-2alpha, but not HIF-1alpha, promotes iron absorption in mice. J Clin Invest 2009; 119: 1159-1166.
-
(2009)
J Clin Invest
, vol.119
, pp. 1159-1166
-
-
Mastrogiannaki, M.1
Matak, P.2
Keith, B.3
Simon, M.C.4
Vaulont, S.5
Peyssonnaux, C.6
-
36
-
-
70349478990
-
MicroRNA-210 controls mitochondrial metabolism during hypoxia by repressing the iron-sulfur cluster assembly proteins ISCU1/2
-
Chan SY, Zhang YY, Hemann C, Mahoney CE, Zweier JL, Loscalzo J. MicroRNA-210 controls mitochondrial metabolism during hypoxia by repressing the iron-sulfur cluster assembly proteins ISCU1/2. Cell Metab 2009; 10: 273-284.
-
(2009)
Cell Metab
, vol.10
, pp. 273-284
-
-
Chan, S.Y.1
Zhang, Y.Y.2
Hemann, C.3
Mahoney, C.E.4
Zweier, J.L.5
Loscalzo, J.6
-
37
-
-
77956402239
-
MicroRNA-210 regulates mitochondrial free radical response to hypoxia and krebs cycle in cancer cells by targeting iron sulfur cluster protein ISCU
-
Favaro E, Ramachandran A, McCormick R, Gee H, Blancher C, Crosby M et al. MicroRNA-210 regulates mitochondrial free radical response to hypoxia and krebs cycle in cancer cells by targeting iron sulfur cluster protein ISCU. PLoS One 2010; 5: e10345.
-
(2010)
PLoS One
, vol.5
-
-
Favaro, E.1
Ramachandran, A.2
McCormick, R.3
Gee, H.4
Blancher, C.5
Crosby, M.6
-
38
-
-
38149059911
-
Mitochondria and cellular oxygen sensing in the HIF pathway
-
Taylor CT. Mitochondria and cellular oxygen sensing in the HIF pathway. Biochem J 2008; 409: 19-26.
-
(2008)
Biochem J
, vol.409
, pp. 19-26
-
-
Taylor, C.T.1
-
39
-
-
76749143747
-
Altered gene expression and DNA damage in peripheral blood cells from Friedreich's ataxia patients: Cellular model of pathology
-
Haugen AC, Di Prospero NA, Parker JS, Fannin RD, Chou J, Meyer JN et al. Altered gene expression and DNA damage in peripheral blood cells from Friedreich's ataxia patients: cellular model of pathology. PLoS Genet 2010; 6: e1000812.
-
(2010)
PLoS Genet
, vol.6
-
-
Haugen, A.C.1
Di Prospero, N.A.2
Parker, J.S.3
Fannin, R.D.4
Chou, J.5
Meyer, J.N.6
-
40
-
-
33845693952
-
Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts
-
DOI 10.1016/j.abb.2006.09.010, PII S000398610600347X
-
Lu C, Cortopassi G. Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts. Arch Biochem Biophys 2007; 457: 111-122. (Pubitemid 44969278)
-
(2007)
Archives of Biochemistry and Biophysics
, vol.457
, Issue.1
, pp. 111-122
-
-
Lu, C.1
Cortopassi, G.2
|