Browsing by Author Pook, MA

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Showing results 17 to 29 of 29 < previous 
Issue DateTitleAuthor(s)
12-Jul-2021Interruptions of the FXN GAA Repeat Tract Delay the Age at Onset of Friedreich’s Ataxia in a Location Dependent MannerNethisinghe, S; Kesavan, M; Ging, H; Labrum, R; Polke, JM; Islam, S; Garcia-Moreno, H; Callaghan, MF; Cavalcanti, F; Pook, MA; Giunti, P
15-Aug-2016Lentivirus-meditated frataxin gene delivery reverses genome instability in Friedreich ataxia patient and mouse model fibroblastsKhonsari, H; Schneider, M; Al-Mahdawi, S; Chianea, YG; Themis, M; Parris, C; Pook, MA; Themis, M
2012The mismatch repair system protects against intergenerational GAA repeat instability in a Friedreich ataxia mouse modelEzzatizadeh, V; Pinto, RM; Sandi, C; Sandi, M; Al-Mahdawi, S; Te Riele, H; Pook, MA
-'Mitochondrial energy imbalance and lipid peroxidation cause cell death in Friedreich's ataxia'Parkinson, MH; Hargreaves, IP; Angelova, PR; Sandi, C; Pook, MA; Giunti, P; Abramov, AY
2014MutLα heterodimers modify the molecular phenotype of Friedreich ataxiaEzzatizadeh, V; Sandi, C; Sandi, M; Anjomani-Virmouni, S; Al-Mahdawi, S; Pook, MA
26-Jan-2023A new FRDA mouse model [Fxnnull:YG8s(GAA) > 800] with more than 800 GAA repeatsKalef-Ezra, E; Edzeamey, FJ; Valle, A; Khonsari, H; Kleine, P; Oggianu, C; Al-Mahdawi, S; Pook, MA; Anjomani Virmouni, S
2012Novel frataxin isoforms may contribute to the pathological mechanism of friedreich ataxiaXia, H; Cao, Y; Dai, X; Marelja, Z; Zhou, D; Mo, R; Al-Mahdawi, S; Pook, MA; Leimkühler, S; Rouault, TA; Li, K
2015A novel GAA-repeat-expansion-based mouse model of Friedreich's ataxia.Anjomani Virmouni, S; Ezzatizadeh, V; Sandi, C; Sandi, M; Al-Mahdawi, S; Chutake, Y; Pook, MA
2013Pharmacological screening using an FXN-EGFP cellular genomic reporter assay for the therapy of Friedreich ataxiaLi, L; Voullaire, L; Sandi, C; Pook, MA; Ioannou, PA; Delatycki, MB; Sarsero, JP
2012Pms2 suppresses large expansions of the (GAA·TTC)n sequence in neuronal tissuesBourn, RL; De Biase, I; Pinto, RM; Sandi, C; Al-Mahdawi, S; Pook, MA; Bidichandani, S
2011Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse modelSandi, C; Pinto, RM; Al-Mahdawi, S; Ezzatizadeh, V; Barnes, G; Jones, S; Rusche, JR; Gottesfeld, JM; Pook, MA
19-Feb-2019The role of mitochondrial labile iron in Friedreich's ataxia skin fibroblasts sensitivity to ultraviolet AReelfs, O; Abbate, V; Cilibrizzi, A; Pook, MA; Hider, RC; Pourzand, C
2015Targeting lipid peroxidation and mitochondrial imbalance in Friedreich's ataxiaAbeti, R; Uzun, E; Renganathan, I; Honda, T; Pook, MA; Giunti, P