Agency
National Institute of Health (NIH)
Institute
National Institute of Neurological Disorders and Stroke (NINDS)
Type
Research Project (R01)
Project #
3R01NS034192-02S1
Application #
2547356
Study Section
Mammalian Genetics Study Section (MGN)
Project Start
1995-09-30
Project End
1998-08-31
Budget Start
1996-09-30
Budget End
1997-08-31
Support Year
2
Fiscal Year
1997
Total Cost
Indirect Cost
Name
University of Montreal
Department
Type
DUNS #
207622838
City
Montreal
State
PQ
Country
Canada
Zip Code
H3 3-J7
Coppola, Giovanni; Marmolino, Daniele; Lu, Daning et al. (2009) Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARgamma pathway as a therapeutic target in Friedreich's ataxia. Hum Mol Genet 18:2452-61
Coppola, Giovanni; Choi, Sang-Hyun; Santos, Manuela M et al. (2006) Gene expression profiling in frataxin deficient mice: microarray evidence for significant expression changes without detectable neurodegeneration. Neurobiol Dis 22:302-11
Miranda, Carlos J; Santos, Manuela M; Ohshima, Keiichi et al. (2004) Frataxin overexpressing mice. FEBS Lett 572:281-8
Santos, Manuela M; Miranda, Carlos J; Levy, Joanne E et al. (2003) Iron metabolism in mice with partial frataxin deficiency. Cerebellum 2:146-53
Santos, M M; Ohshima, K; Pandolfo, M (2001) Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm. Hum Mol Genet 10:1935-44
Jiralerspong, S; Ge, B; Hudson, T J et al. (2001) Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells. FEBS Lett 509:101-5
Sakamoto, N; Larson, J E; Iyer, R R et al. (2001) GGA*TCC-interrupted triplets in long GAA*TTC repeats inhibit the formation of triplex and sticky DNA structures, alleviate transcription inhibition, and reduce genetic instabilities. J Biol Chem 276:27178-87
Sakamoto, N; Ohshima, K; Montermini, L et al. (2001) Sticky DNA, a self-associated complex formed at long GAA*TTC repeats in intron 1 of the frataxin gene, inhibits transcription. J Biol Chem 276:27171-7
Labuda, M; Labuda, D; Miranda, C et al. (2000) Unique origin and specific ethnic distribution of the Friedreich ataxia GAA expansion. Neurology 54:2322-4
Cossee, M; Durr, A; Schmitt, M et al. (1999) Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes. Ann Neurol 45:200-6

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