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Results: 1 to 20 of 99

1.

Gene expression profiling in frataxin deficient mice: microarray evidence for significant expression changes without detectable neurodegeneration.

Coppola G, Choi SH, Santos MM, Miranda CJ, Tentler D, Wexler EM, Pandolfo M, Geschwind DH.

Neurobiol Dis. 2006 May;22(2):302-11. Epub 2006 Jan 25.

PMID:
16442805
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

Functional recovery in a Friedreich's ataxia mouse model by frataxin gene transfer using an HSV-1 amplicon vector.

Lim F, Palomo GM, Mauritz C, Giménez-Cassina A, Illana B, Wandosell F, Díaz-Nido J.

Mol Ther. 2007 Jun;15(6):1072-8. Epub 2007 Mar 20.

PMID:
17375064
[PubMed - indexed for MEDLINE]
3.

Friedreich's ataxia and frataxin: molecular genetics, evolution and pathogenesis (Review).

Palau F.

Int J Mol Med. 2001 Jun;7(6):581-9. Review.

PMID:
11351269
[PubMed - indexed for MEDLINE]
4.

Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia.

Simon D, Seznec H, Gansmuller A, Carelle N, Weber P, Metzger D, Rustin P, Koenig M, Puccio H.

J Neurosci. 2004 Feb 25;24(8):1987-95.

PMID:
14985441
[PubMed - indexed for MEDLINE]
Free Article
5.

Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis.

Pook MA, Al-Mahdawi S, Carroll CJ, Cossée M, Puccio H, Lawrence L, Clark P, Lowrie MB, Bradley JL, Cooper JM, Koenig M, Chamberlain S.

Neurogenetics. 2001 Oct;3(4):185-93.

PMID:
11714098
[PubMed - indexed for MEDLINE]
6.

HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model.

Rai M, Soragni E, Jenssen K, Burnett R, Herman D, Coppola G, Geschwind DH, Gottesfeld JM, Pandolfo M.

PLoS One. 2008 Apr 9;3(4):e1958. doi: 10.1371/journal.pone.0001958.

PMID:
18463734
[PubMed - indexed for MEDLINE]
Free PMC Article
7.

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
[PubMed - indexed for MEDLINE]
8.

GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology.

Al-Mahdawi S, Pinto RM, Varshney D, Lawrence L, Lowrie MB, Hughes S, Webster Z, Blake J, Cooper JM, King R, Pook MA.

Genomics. 2006 Nov;88(5):580-90. Epub 2006 Aug 17.

PMID:
16919418
[PubMed - indexed for MEDLINE]
Free PMC Article
9.

Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant.

Huang ML, Becker EM, Whitnall M, Suryo Rahmanto Y, Ponka P, Richardson DR.

Proc Natl Acad Sci U S A. 2009 Sep 22;106(38):16381-6. doi: 10.1073/pnas.0906784106. Epub 2009 Sep 4.

PMID:
19805308
[PubMed - indexed for MEDLINE]
Free PMC Article
10.

Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia.

Calabrese V, Lodi R, Tonon C, D'Agata V, Sapienza M, Scapagnini G, Mangiameli A, Pennisi G, Stella AM, Butterfield DA.

J Neurol Sci. 2005 Jun 15;233(1-2):145-62. Review.

PMID:
15896810
[PubMed - indexed for MEDLINE]
11.

Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARgamma pathway as a therapeutic target in Friedreich's ataxia.

Coppola G, Marmolino D, Lu D, Wang Q, Cnop M, Rai M, Acquaviva F, Cocozza S, Pandolfo M, Geschwind DH.

Hum Mol Genet. 2009 Jul 1;18(13):2452-61. doi: 10.1093/hmg/ddp183. Epub 2009 Apr 17.

PMID:
19376812
[PubMed - indexed for MEDLINE]
Free PMC Article
12.

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.

PMID:
19703283
[PubMed - indexed for MEDLINE]
Free PMC Article
13.

Glutathione-dependent redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia.

Auchère F, Santos R, Planamente S, Lesuisse E, Camadro JM.

Hum Mol Genet. 2008 Sep 15;17(18):2790-802. doi: 10.1093/hmg/ddn178. Epub 2008 Jun 18.

PMID:
18562474
[PubMed - indexed for MEDLINE]
Free Article
14.

Altered lipid metabolism in a Drosophila model of Friedreich's ataxia.

Navarro JA, Ohmann E, Sanchez D, Botella JA, Liebisch G, Moltó MD, Ganfornina MD, Schmitz G, Schneuwly S.

Hum Mol Genet. 2010 Jul 15;19(14):2828-40. doi: 10.1093/hmg/ddq183. Epub 2010 May 10.

PMID:
20460268
[PubMed - indexed for MEDLINE]
Free Article
15.

Partial correction of sensitivity to oxidant stress in Friedreich ataxia patient fibroblasts by frataxin-encoding adeno-associated virus and lentivirus vectors.

Fleming J, Spinoulas A, Zheng M, Cunningham SC, Ginn SL, McQuilty RC, Rowe PB, Alexander IE.

Hum Gene Ther. 2005 Aug;16(8):947-56.

PMID:
16076253
[PubMed - indexed for MEDLINE]
16.

Multicellular models of Friedreich ataxia.

Puccio H.

J Neurol. 2009 Mar;256 Suppl 1:18-24. doi: 10.1007/s00415-009-1004-1. Review.

PMID:
19283346
[PubMed - indexed for MEDLINE]
17.

Friedreich's ataxia.

Alper G, Narayanan V.

Pediatr Neurol. 2003 May;28(5):335-41. Review.

PMID:
12878293
[PubMed - indexed for MEDLINE]
18.

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits.

Puccio H, Simon D, Cossée M, Criqui-Filipe P, Tiziano F, Melki J, Hindelang C, Matyas R, Rustin P, Koenig M.

Nat Genet. 2001 Feb;27(2):181-6.

PMID:
11175786
[PubMed - indexed for MEDLINE]
19.

A gene expression phenotype in lymphocytes from Friedreich ataxia patients.

Coppola G, Burnett R, Perlman S, Versano R, Gao F, Plasterer H, Rai M, Saccá F, Filla A, Lynch DR, Rusche JR, Gottesfeld JM, Pandolfo M, Geschwind DH.

Ann Neurol. 2011 Nov;70(5):790-804. doi: 10.1002/ana.22526.

PMID:
22162061
[PubMed - indexed for MEDLINE]
Free PMC Article
20.

Human BAC-mediated rescue of the Friedreich ataxia knockout mutation in transgenic mice.

Sarsero JP, Li L, Holloway TP, Voullaire L, Gazeas S, Fowler KJ, Kirby DM, Thorburn DR, Galle A, Cheema S, Koenig M, Williamson R, Ioannou PA.

Mamm Genome. 2004 May;15(5):370-82.

PMID:
15170226
[PubMed - indexed for MEDLINE]

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