Browsing by Author Pook, MA

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Issue DateTitleAuthor(s)
2018Bone marrow transplantation stimulates neural repair in Friedreich's ataxia miceKemp, KC; Hares, K; Redondo, J; Cook, AJ; Haynes, HR; Burton, BR; Pook, MA; Rice, CM; Scolding, NJ; Wilkins, A
2014Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich AtaxiaVirmouni, SA; Sandi, C; Al-Mahdawi, S; Pook, MA
2015Characterization of the retinal pigment epithelium in Friedreich ataxiaCrombie, DE; Van Bergen, N; Davidson, KC; Anjomani Virmouni, S; Mckelvie, PA; Chrysostomou, V; Conquest, A; Corben, LA; Pook, MA; Kulkarni, T; Trounce, IA; Pera, MF; Delatycki, MB; Pébay, A
2013Comparative (computational) analysis of the DNA methylation status of trinucleotide repeat expansion diseasesGhorbani, M; Taylor, SJE; Pook, MA; Payne, A
2014Dyclonine 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-2018Effect of diazoxide on Friedreich ataxia modelsSantoro, 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
2014The emerging role of 5-hydroxymethylcytosine in neurodegenerative diseasesAl-Mahdawi, S; Virmouni, SA; Pook, MA
2014Epigenetic-based therapies for Friedreich ataxiaSandi, C; Sandi, M; Virmouni, SA; Al-Mahdawi, S; Pook, MA
2013Epigenetics in Friedreich's ataxia: Challenges and opportunities for therapySandi, C; Al-Mahdawi, S; Pook, MA
13-Jun-2022Frataxin Deficit Leads to Reduced Dynamics of Growth Cones in Dorsal Root Ganglia Neurons of Friedreich’s Ataxia YG8sR Model: A Multilinear Algebra ApproachMollá, B; Sáenz-Gamboa, JJ; Insuasty, E; de la Iglesia-Vaya, M; Pook, MA; Pallardó, FV; Palau, F; Gonzalez-Cabo, P
2007The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissuesPook, MA; Al-Mahdawi, S; Mouro Pinto, R; Sandi, C; Trabzuni, D
2013Friedreich ataxia patient tissues exhibit increased 5-hydroxymethylcytosine modification and decreased CTCF binding at the FXN locusAl-Mahdawi, S; Sandi, C; Mouro Pinto, R; Pook, MA
2015FXN promoter silencing in the humanized mouse model of Friedreich AtaxiaChutake, YK; Costello, WN; Lam, CC; Parikh, AC; Hughes, TT; Michalopulos, MG; Pook, MA; Bidichandani, SI
2006GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathologyPook, MA; Al-Mahdawi, S; Mouro Pinto, R
2006The 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 modelPook, MA; Clark, RM; De Biase, I; Al-Mahdawi, S; Malykhina, AP; Bidichandani, S
2014Generation and characterisation of Friedreich ataxia YG8R mouse fibroblast and neural stem cell modelsSandi, C; Sandi, M; Jassal, H; Ezzatizadeh, V; Anjomani-Virmouni, S; Al-Mahdawi, S; Pook, MA
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