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Items: 1 to 20 of 216

1.

Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia.

Vaubel RA, Isaya G.

Mol Cell Neurosci. 2013 Jul;55:50-61. doi: 10.1016/j.mcn.2012.08.003. Epub 2012 Aug 11. Review.

2.

Iron metabolism and mitochondrial abnormalities in Friedreich ataxia.

Pandolfo M.

Blood Cells Mol Dis. 2002 Nov-Dec;29(3):536-47; discussion 548-52. Review.

PMID:
12547248
3.

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia.

Rötig A, de Lonlay P, Chretien D, Foury F, Koenig M, Sidi D, Munnich A, Rustin P.

Nat Genet. 1997 Oct;17(2):215-7.

PMID:
9326946
4.

Normal and Friedreich ataxia cells express different isoforms of frataxin with complementary roles in iron-sulfur cluster assembly.

Gakh O, Bedekovics T, Duncan SF, Smith DY 4th, Berkholz DS, Isaya G.

J Biol Chem. 2010 Dec 3;285(49):38486-501. doi: 10.1074/jbc.M110.145144. Epub 2010 Oct 2. Erratum in: J Biol Chem. 2011 Mar 11;286(10):8708.

5.

Friedreich ataxia: a paradigm for mitochondrial diseases.

Puccio H, Koenig M.

Curr Opin Genet Dev. 2002 Jun;12(3):272-7. Review.

PMID:
12076669
6.

Missense mutations linked to friedreich ataxia have different but synergistic effects on mitochondrial frataxin isoforms.

Li H, Gakh O, Smith DY 4th, Ranatunga WK, Isaya G.

J Biol Chem. 2013 Feb 8;288(6):4116-27. doi: 10.1074/jbc.M112.435263. Epub 2012 Dec 26.

7.

Frataxin and mitochondrial FeS cluster biogenesis.

Stemmler TL, Lesuisse E, Pain D, Dancis A.

J Biol Chem. 2010 Aug 27;285(35):26737-43. doi: 10.1074/jbc.R110.118679. Epub 2010 Jun 3. Review.

8.

Neurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stress.

Gomes CM, Santos R.

Oxid Med Cell Longev. 2013;2013:487534. doi: 10.1155/2013/487534. Epub 2013 Jul 9. Review.

9.

Therapeutic developments in Friedreich ataxia.

Wilson RB.

J Child Neurol. 2012 Sep;27(9):1212-6. doi: 10.1177/0883073812449691. Epub 2012 Jul 12. Review.

10.

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, Rustin P, Koenig M, Puccio H.

BMC Neurol. 2009 Aug 24;9:46. doi: 10.1186/1471-2377-9-46.

11.

Frataxin and the molecular mechanism of mitochondrial iron-loading in Friedreich's ataxia.

Chiang S, Kovacevic Z, Sahni S, Lane DJ, Merlot AM, Kalinowski DS, Huang ML, Richardson DR.

Clin Sci (Lond). 2016 Jun 1;130(11):853-70. doi: 10.1042/CS20160072. Review.

PMID:
27129098
12.

Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones.

Shan Y, Napoli E, Cortopassi G.

Hum Mol Genet. 2007 Apr 15;16(8):929-41. Epub 2007 Mar 1.

PMID:
17331979
13.

Friedreich ataxia: the oxidative stress paradox.

Seznec H, Simon D, Bouton C, Reutenauer L, Hertzog A, Golik P, Procaccio V, Patel M, Drapier JC, Koenig M, Puccio H.

Hum Mol Genet. 2005 Feb 15;14(4):463-74. Epub 2004 Dec 22.

PMID:
15615771
14.

Iron dysregulation in Friedreich ataxia.

Wilson RB.

Semin Pediatr Neurol. 2006 Sep;13(3):166-75. Review.

PMID:
17101455
15.

Friedreich's ataxia: past, present and future.

Marmolino D.

Brain Res Rev. 2011 Jun 24;67(1-2):311-30. doi: 10.1016/j.brainresrev.2011.04.001. Epub 2011 Apr 17. Review.

PMID:
21550666
16.

A dynamic model of the proteins that form the initial iron-sulfur cluster biogenesis machinery in yeast mitochondria.

Amela I, Delicado P, Gómez A, Querol E, Cedano J.

Protein J. 2013 Mar;32(3):183-96. doi: 10.1007/s10930-013-9475-4.

PMID:
23463383
17.

No changes in heme synthesis in human Friedreich´s ataxia erythroid progenitor cells.

Steinkellner H, Singh HN, Muckenthaler MU, Goldenberg H, Moganty RR, Scheiber-Mojdehkar B, Sturm B.

Gene. 2017 Jul 20;621:5-11. doi: 10.1016/j.gene.2017.04.014. Epub 2017 Apr 12.

PMID:
28412459
18.

Human frataxin maintains mitochondrial iron homeostasis in Saccharomyces cerevisiae.

Cavadini P, Gellera C, Patel PI, Isaya G.

Hum Mol Genet. 2000 Oct 12;9(17):2523-30.

PMID:
11030757
19.

Changes in mitochondrial glutathione levels and protein thiol oxidation in ∆yfh1 yeast cells and the lymphoblasts of patients with Friedreich's ataxia.

Bulteau AL, Planamente S, Jornea L, Dur A, Lesuisse E, Camadro JM, Auchère F.

Biochim Biophys Acta. 2012 Feb;1822(2):212-25. doi: 10.1016/j.bbadis.2011.11.003. Epub 2011 Nov 11.

20.

The role of frataxin in fission yeast iron metabolism: implications for Friedreich's ataxia.

Wang Y, Wang Y, Marcus S, Busenlehner LS.

Biochim Biophys Acta. 2014 Oct;1840(10):3022-33. doi: 10.1016/j.bbagen.2014.06.017. Epub 2014 Jul 3.

PMID:
24997422

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