Showing results 14 to 29 of 29
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Issue Date | Title | Author(s) |
2006 | GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology | Pook, MA; Al-Mahdawi, S; Mouro Pinto, R |
2006 | The GAA triplet-repeat is unstable in the context of the human FXN locus and displays age-dependent expansions in cerebellum and DRG in a transgenic mouse model | Pook, MA; Clark, RM; De Biase, I; Al-Mahdawi, S; Malykhina, AP; Bidichandani, S |
2014 | Generation and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell models | Sandi, C; Sandi, M; Jassal, H; Ezzatizadeh, V; Anjomani-Virmouni, S; Al-Mahdawi, S; Pook, MA |
12-Jul-2021 | Interruptions of the FXN GAA Repeat Tract Delay the Age at Onset of Friedreich’s Ataxia in a Location Dependent Manner | Nethisinghe, S; Kesavan, M; Ging, H; Labrum, R; Polke, JM; Islam, S; Garcia-Moreno, H; Callaghan, MF; Cavalcanti, F; Pook, MA; Giunti, P |
15-Aug-2016 | Lentivirus-meditated frataxin gene delivery reverses genome instability in Friedreich ataxia patient and mouse model fibroblasts | Khonsari, H; Schneider, M; Al-Mahdawi, S; Chianea, YG; Themis, M; Parris, C; Pook, MA; Themis, M |
2012 | The mismatch repair system protects against intergenerational GAA repeat instability in a Friedreich ataxia mouse model | Ezzatizadeh, 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 |
2014 | MutLα heterodimers modify the molecular phenotype of Friedreich ataxia | Ezzatizadeh, V; Sandi, C; Sandi, M; Anjomani-Virmouni, S; Al-Mahdawi, S; Pook, MA |
26-Jan-2023 | A new FRDA mouse model [Fxnnull:YG8s(GAA) > 800] with more than 800 GAA repeats | Kalef-Ezra, E; Edzeamey, FJ; Valle, A; Khonsari, H; Kleine, P; Oggianu, C; Al-Mahdawi, S; Pook, MA; Anjomani Virmouni, S |
2012 | Novel frataxin isoforms may contribute to the pathological mechanism of friedreich ataxia | Xia, H; Cao, Y; Dai, X; Marelja, Z; Zhou, D; Mo, R; Al-Mahdawi, S; Pook, MA; Leimkühler, S; Rouault, TA; Li, K |
2015 | A 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 |
2013 | 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 |
2012 | Pms2 suppresses large expansions of the (GAA·TTC)n sequence in neuronal tissues | Bourn, RL; De Biase, I; Pinto, RM; Sandi, C; Al-Mahdawi, S; Pook, MA; Bidichandani, S |
2011 | Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model | Sandi, C; Pinto, RM; Al-Mahdawi, S; Ezzatizadeh, V; Barnes, G; Jones, S; Rusche, JR; Gottesfeld, JM; Pook, MA |
19-Feb-2019 | The role of mitochondrial labile iron in Friedreich's ataxia skin fibroblasts sensitivity to ultraviolet A | Reelfs, O; Abbate, V; Cilibrizzi, A; Pook, MA; Hider, RC; Pourzand, C |
2015 | Targeting lipid peroxidation and mitochondrial imbalance in Friedreich's ataxia | Abeti, R; Uzun, E; Renganathan, I; Honda, T; Pook, MA; Giunti, P |