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SINEUP non-coding RNAs rescue defective frataxin expression and activity in a cellular model of Friedreich's Ataxia.
Bon C, Luffarelli R, Russo R, Fortuni S, Pierattini B, Santulli C, Fimiani C, Persichetti F, Cotella D, Mallamaci A, Santoro C, Carninci P, Espinoza S, Testi R, Zucchelli S, Condò I, Gustincich S. Bon C, et al. Among authors: testi r. Nucleic Acids Res. 2019 Nov 18;47(20):10728-10743. doi: 10.1093/nar/gkz798. Nucleic Acids Res. 2019. PMID: 31584077 Free PMC article.
A pool of extramitochondrial frataxin that promotes cell survival.
Condò I, Ventura N, Malisan F, Tomassini B, Testi R. Condò I, et al. Among authors: testi r. J Biol Chem. 2006 Jun 16;281(24):16750-6. doi: 10.1074/jbc.M511960200. Epub 2006 Apr 11. J Biol Chem. 2006. PMID: 16608849 Free article.
In vivo maturation of human frataxin.
Condò I, Ventura N, Malisan F, Rufini A, Tomassini B, Testi R. Condò I, et al. Among authors: testi r. Hum Mol Genet. 2007 Jul 1;16(13):1534-40. doi: 10.1093/hmg/ddm102. Epub 2007 Apr 27. Hum Mol Genet. 2007. PMID: 17468497
Highly specific ubiquitin-competing molecules effectively promote frataxin accumulation and partially rescue the aconitase defect in Friedreich ataxia cells.
Rufini A, Cavallo F, Condò I, Fortuni S, De Martino G, Incani O, Di Venere A, Benini M, Massaro DS, Arcuri G, Serio D, Malisan F, Testi R. Rufini A, et al. Among authors: testi r. Neurobiol Dis. 2015 Mar;75:91-9. doi: 10.1016/j.nbd.2014.12.011. Epub 2014 Dec 27. Neurobiol Dis. 2015. PMID: 25549872 Free PMC article.
Src inhibitors modulate frataxin protein levels.
Cherubini F, Serio D, Guccini I, Fortuni S, Arcuri G, Condò I, Rufini A, Moiz S, Camerini S, Crescenzi M, Testi R, Malisan F. Cherubini F, et al. Among authors: testi r. Hum Mol Genet. 2015 Aug 1;24(15):4296-305. doi: 10.1093/hmg/ddv162. Epub 2015 May 6. Hum Mol Genet. 2015. PMID: 25948553
168 results