252 related articles for article (PubMed ID: 19703283)
1. 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; 9():46. PubMed ID: 19703283
[TBL] [Abstract][Full Text] [Related]
2. GRP75 overexpression rescues frataxin deficiency and mitochondrial phenotypes in Friedreich ataxia cellular models.
Dong YN; McMillan E; Clark EM; Lin H; Lynch DR
Hum Mol Genet; 2019 May; 28(10):1594-1607. PubMed ID: 30590615
[TBL] [Abstract][Full Text] [Related]
3. The first cellular models based on frataxin missense mutations that reproduce spontaneously the defects associated with Friedreich ataxia.
Calmels N; Schmucker S; Wattenhofer-Donzé M; Martelli A; Vaucamps N; Reutenauer L; Messaddeq N; Bouton C; Koenig M; Puccio H
PLoS One; 2009 Jul; 4(7):e6379. PubMed ID: 19629184
[TBL] [Abstract][Full Text] [Related]
4. Primary and secondary drug screening assays for Friedreich ataxia.
Cotticelli MG; Rasmussen L; Kushner NL; McKellip S; Sosa MI; Manouvakhova A; Feng S; White EL; Maddry JA; Heemskerk J; Oldt RJ; Surrey LF; Ochs R; Wilson RB
J Biomol Screen; 2012 Mar; 17(3):303-13. PubMed ID: 22086726
[TBL] [Abstract][Full Text] [Related]
5. Posttranslational regulation of mitochondrial frataxin and identification of compounds that increase frataxin levels in Friedreich's ataxia.
Hackett PT; Jia X; Li L; Ward DM
J Biol Chem; 2022 Jun; 298(6):101982. PubMed ID: 35472330
[TBL] [Abstract][Full Text] [Related]
6. PPAR gamma agonist leriglitazone improves frataxin-loss impairments in cellular and animal models of Friedreich Ataxia.
Rodríguez-Pascau L; Britti E; Calap-Quintana P; Dong YN; Vergara C; Delaspre F; Medina-Carbonero M; Tamarit J; Pallardó FV; Gonzalez-Cabo P; Ros J; Lynch DR; Martinell M; Pizcueta P
Neurobiol Dis; 2021 Jan; 148():105162. PubMed ID: 33171227
[TBL] [Abstract][Full Text] [Related]
7. Iron-sulfur cluster synthesis, iron homeostasis and oxidative stress in Friedreich ataxia.
Vaubel RA; Isaya G
Mol Cell Neurosci; 2013 Jul; 55():50-61. PubMed ID: 22917739
[TBL] [Abstract][Full Text] [Related]
8. Phenotypic Screening for Friedreich Ataxia Using Random shRNA Selection.
Cotticelli MG; Acquaviva F; Xia S; Kaur A; Wang Y; Wilson RB
J Biomol Screen; 2015 Oct; 20(9):1084-90. PubMed ID: 26286937
[TBL] [Abstract][Full Text] [Related]
9. Pharmacological screening using an FXN-EGFP cellular genomic reporter assay for the therapy of Friedreich ataxia.
Li L; Voullaire L; Sandi C; Pook MA; Ioannou PA; Delatycki MB; Sarsero JP
PLoS One; 2013; 8(2):e55940. PubMed ID: 23418481
[TBL] [Abstract][Full Text] [Related]
10. 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; 16(8):929-41. PubMed ID: 17331979
[TBL] [Abstract][Full Text] [Related]
11. 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; Berkholz DS; Isaya G
J Biol Chem; 2010 Dec; 285(49):38486-501. PubMed ID: 20889968
[TBL] [Abstract][Full Text] [Related]
12. Mechanisms of Mitochondrial Iron-Sulfur Protein Biogenesis.
Lill R; Freibert SA
Annu Rev Biochem; 2020 Jun; 89():471-499. PubMed ID: 31935115
[TBL] [Abstract][Full Text] [Related]
13. 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. PubMed ID: 11175786
[TBL] [Abstract][Full Text] [Related]
14. Frataxin expression rescues mitochondrial dysfunctions in FRDA cells.
Tan G; Chen LS; Lonnerdal B; Gellera C; Taroni FA; Cortopassi GA
Hum Mol Genet; 2001 Sep; 10(19):2099-107. PubMed ID: 11590127
[TBL] [Abstract][Full Text] [Related]
15. Frataxin and mitochondrial FeS cluster biogenesis.
Stemmler TL; Lesuisse E; Pain D; Dancis A
J Biol Chem; 2010 Aug; 285(35):26737-26743. PubMed ID: 20522547
[TBL] [Abstract][Full Text] [Related]
16. Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells.
Tan G; Napoli E; Taroni F; Cortopassi G
Hum Mol Genet; 2003 Jul; 12(14):1699-711. PubMed ID: 12837693
[TBL] [Abstract][Full Text] [Related]
17. Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia.
Igoillo-Esteve M; Oliveira AF; Cosentino C; Fantuzzi F; Demarez C; Toivonen S; Hu A; Chintawar S; Lopes M; Pachera N; Cai Y; Abdulkarim B; Rai M; Marselli L; Marchetti P; Tariq M; Jonas JC; Boscolo M; Pandolfo M; Eizirik DL; Cnop M
JCI Insight; 2020 Jan; 5(2):. PubMed ID: 31877117
[TBL] [Abstract][Full Text] [Related]
18. Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia.
Seznec H; Simon D; Monassier L; Criqui-Filipe P; Gansmuller A; Rustin P; Koenig M; Puccio H
Hum Mol Genet; 2004 May; 13(10):1017-24. PubMed ID: 15028670
[TBL] [Abstract][Full Text] [Related]
19. Friedreich ataxia: a paradigm for mitochondrial diseases.
Puccio H; Koenig M
Curr Opin Genet Dev; 2002 Jun; 12(3):272-7. PubMed ID: 12076669
[TBL] [Abstract][Full Text] [Related]
20. Drug repositioning screening identifies etravirine as a potential therapeutic for friedreich's ataxia.
Alfedi G; Luffarelli R; Condò I; Pedini G; Mannucci L; Massaro DS; Benini M; Toschi N; Alaimo G; Panarello L; Pacini L; Fortuni S; Serio D; Malisan F; Testi R; Rufini A
Mov Disord; 2019 Mar; 34(3):323-334. PubMed ID: 30624801
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
