Frataxin deficiency leads to defects in expression of antioxidants and Nrf2 expression in dorsal root ganglia of the Friedreich's ataxia YG8R mouse model
Shan, Yuxi, Schoenfeld, Robert A, Hayashi, Genki, Napoli, Eleonora, Akiyama, Tasuku, Iodi Carstens, Mirela, Carstens, Earl E, Pook, Mark A, Cortopassi, Gino A
Published in Antioxidants & redox signaling (01.11.2013)
Published in Antioxidants & redox signaling (01.11.2013)
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Interruptions of the FXN GAA Repeat Tract Delay the Age at Onset of Friedreich’s Ataxia in a Location Dependent Manner
Nethisinghe, Suran, Kesavan, Maheswaran, Ging, Heather, Labrum, Robyn, Polke, James M., Islam, Saiful, Garcia-Moreno, Hector, Callaghan, Martina F., Cavalcanti, Francesca, Pook, Mark A., Giunti, Paola
Published in International journal of molecular sciences (13.07.2021)
Published in International journal of molecular sciences (13.07.2021)
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A new FRDA mouse model [ Fxn null :YG8s(GAA) > 800] with more than 800 GAA repeats
Kalef-Ezra, Ester, Edzeamey, Fred Jonathan, Valle, Adamo, Khonsari, Hassan, Kleine, Paula, Oggianu, Carlo, Al-Mahdawi, Sahar, Pook, Mark A, Anjomani Virmouni, Sara
Published in Frontiers in neuroscience (26.01.2023)
Published in Frontiers in neuroscience (26.01.2023)
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Prolonged treatment with pimelic o -aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model
Sandi, Chiranjeevi, Pinto, Ricardo Mouro, Al-Mahdawi, Sahar, Ezzatizadeh, Vahid, Barnes, Glenn, Jones, Steve, Rusche, James R, Gottesfeld, Joel M, Pook, Mark A
Published in Neurobiology of disease (01.06.2011)
Published in Neurobiology of disease (01.06.2011)
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Cellular, molecular and functional characterisation of YAC transgenic mouse models of Friedreich ataxia
Anjomani Virmouni, Sara, Sandi, Chiranjeevi, Al-Mahdawi, Sahar, Pook, Mark A
Published in PloS one (08.09.2014)
Published in PloS one (08.09.2014)
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Large Interruptions of GAA Repeat Expansion Mutations in Friedreich Ataxia Are Very Rare
Al-Mahdawi, Sahar, Ging, Heather, Bayot, Aurelien, Cavalcanti, Francesca, La Cognata, Valentina, Cavallaro, Sebastiano, Giunti, Paola, Pook, Mark A
Published in Frontiers in cellular neuroscience (21.11.2018)
Published in Frontiers in cellular neuroscience (21.11.2018)
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MutLα heterodimers modify the molecular phenotype of Friedreich ataxia
Ezzatizadeh, Vahid, Sandi, Chiranjeevi, Sandi, Madhavi, Anjomani-Virmouni, Sara, Al-Mahdawi, Sahar, Pook, Mark A
Published in PloS one (27.06.2014)
Published in PloS one (27.06.2014)
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Novel frataxin isoforms may contribute to the pathological mechanism of Friedreich ataxia
Xia, Haiyan, Cao, Yun, Dai, Xiaoman, Marelja, Zvonimir, Zhou, Di, Mo, Ran, Al-Mahdawi, Sahar, Pook, Mark A, Leimkühler, Silke, Rouault, Tracey A, Li, Kuanyu
Published in PloS one (17.10.2012)
Published in PloS one (17.10.2012)
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Frataxin Deficit Leads to Reduced Dynamics of Growth Cones in Dorsal Root Ganglia Neurons of Friedreich’s Ataxia YG8sR Model: A Multilinear Algebra Approach
Muñoz-Lasso, Diana C., Mollá, Belén, Sáenz-Gamboa, Jhon J., Insuasty, Edwin, de la Iglesia-Vaya, Maria, Pook, Mark A., Pallardó, Federico V., Palau, Francesc, Gonzalez-Cabo, Pilar
Published in Frontiers in molecular neuroscience (13.06.2022)
Published in Frontiers in molecular neuroscience (13.06.2022)
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The mismatch repair system protects against intergenerational GAA repeat instability in a Friedreich ataxia mouse model
Ezzatizadeh, Vahid, Pinto, Ricardo Mouro, Sandi, Chiranjeevi, Sandi, Madhavi, Al-Mahdawi, Sahar, te Riele, Hein, Pook, Mark A
Published in Neurobiology of disease (01.04.2012)
Published in Neurobiology of disease (01.04.2012)
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Pms2 suppresses large expansions of the (GAA·TTC)n sequence in neuronal tissues
Bourn, Rebecka L, De Biase, Irene, Pinto, Ricardo Mouro, Sandi, Chiranjeevi, Al-Mahdawi, Sahar, Pook, Mark A, Bidichandani, Sanjay I
Published in PloS one (11.10.2012)
Published in PloS one (11.10.2012)
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Pharmacological screening using an FXN-EGFP cellular genomic reporter assay for the therapy of Friedreich ataxia
Li, Lingli, Voullaire, Lucille, Sandi, Chiranjeevi, Pook, Mark A, Ioannou, Panos A, Delatycki, Martin B, Sarsero, Joseph P
Published in PloS one (13.02.2013)
Published in PloS one (13.02.2013)
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Targeting lipid peroxidation and mitochondrial imbalance in Friedreich's ataxia
Abeti, Rosella, Uzun, Ebru, Renganathan, Indhushri, Honda, Tadashi, Pook, Mark A., Giunti, Paola
Published in Pharmacological research (01.09.2015)
Published in Pharmacological research (01.09.2015)
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Animal and cellular models of Friedreich ataxia
Perdomini, Morgane, Hick, Aurore, Puccio, Hélène, Pook, Mark A.
Published in Journal of neurochemistry (01.08.2013)
Published in Journal of neurochemistry (01.08.2013)
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Epigenetics in Friedreich's Ataxia: Challenges and Opportunities for Therapy
Sandi, Chiranjeevi, Al-Mahdawi, Sahar, Pook, Mark A
Published in Genetics Research International (01.01.2013)
Published in Genetics Research International (01.01.2013)
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