Showing results 5 to 24 of 29
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Issue Date | Title | Author(s) |
2014 | Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia. | Sahdeo, S; Scott, BD; McMackin, MZ; Jasoliya, M; Brown, B; Wulff, H; Perlman, SL; Pook, MA; Cortopassi, GA |
8-Jan-2018 | Effect of diazoxide on Friedreich ataxia models | Santoro, A; Virmouni, SA; Paradies, E; Coa, VLV; Al-Mahdawi, S; Khoo, M; Porcelli, V; Vozza, A; Perrone, M; Denora, N; Taroni, F; Merla, G; Palmieri, L; Pook, MA; Marobbio, CMT |
2014 | The emerging role of 5-hydroxymethylcytosine in neurodegenerative diseases | Al-Mahdawi, S; Virmouni, SA; Pook, MA |
2014 | Epigenetic-based therapies for Friedreich ataxia | Sandi, C; Sandi, M; Virmouni, SA; Al-Mahdawi, S; Pook, MA |
2013 | Epigenetics in Friedreich's ataxia: Challenges and opportunities for therapy | Sandi, C; Al-Mahdawi, S; Pook, MA |
13-Jun-2022 | Frataxin Deficit Leads to Reduced Dynamics of Growth Cones in Dorsal Root Ganglia Neurons of Friedreich’s Ataxia YG8sR Model: A Multilinear Algebra Approach | Mollá, B; Sáenz-Gamboa, JJ; Insuasty, E; de la Iglesia-Vaya, M; Pook, MA; Pallardó, FV; Palau, F; Gonzalez-Cabo, P |
2007 | The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues | Pook, MA; Al-Mahdawi, S; Mouro Pinto, R; Sandi, C; Trabzuni, D |
2013 | Friedreich ataxia patient tissues exhibit increased 5-hydroxymethylcytosine modification and decreased CTCF binding at the FXN locus | Al-Mahdawi, S; Sandi, C; Mouro Pinto, R; Pook, MA |
2015 | FXN promoter silencing in the humanized mouse model of Friedreich Ataxia | Chutake, YK; Costello, WN; Lam, CC; Parikh, AC; Hughes, TT; Michalopulos, MG; Pook, MA; Bidichandani, SI |
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 |