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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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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Journal Article
The emerging role of 5-hydroxymethylcytosine in neurodegenerative diseases
Al-Mahdawi, Sahar, Virmouni, Sara Anjomani, Pook, Mark A
Published in Frontiers in neuroscience (05.12.2014)
Published in Frontiers in neuroscience (05.12.2014)
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Journal Article
Dimethyl fumarate dosing in humans increases frataxin expression: A potential therapy for Friedreich's Ataxia
Jasoliya, Mittal, Sacca, Francesco, Sahdeo, Sunil, Chedin, Frederic, Pane, Chiara, Brescia Morra, Vincenzo, Filla, Alessandro, Pook, Mark, Cortopassi, Gino
Published in PloS one (03.06.2019)
Published in PloS one (03.06.2019)
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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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Journal Article
Dyclonine rescues frataxin deficiency in animal models and buccal cells of patients with Friedreich's ataxia
Sahdeo, Sunil, Scott, Brian D, McMackin, Marissa Z, Jasoliya, Mittal, Brown, Brandon, Wulff, Heike, Perlman, Susan L, Pook, Mark A, Cortopassi, Gino A
Published in Human molecular genetics (20.12.2014)
Published in Human molecular genetics (20.12.2014)
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Journal Article
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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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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Journal Article
Deranged Calcium Signaling and Neurodegeneration in Spinocerebellar Ataxia Type 3
Chen, Xi, Tang, Tie-Shan, Tu, Huiping, Nelson, Omar, Pook, Mark, Hammer, Robert, Nukina, Nobuyuki, Bezprozvanny, Ilya
Published in The Journal of neuroscience (26.11.2008)
Published in The Journal of neuroscience (26.11.2008)
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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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Journal Article
FAST-1 antisense RNA epigenetically alters FXN expression
Mikaeili, Hajar, Sandi, Madhavi, Bayot, Aurélien, Al-Mahdawi, Sahar, Pook, Mark A.
Published in Scientific reports (21.11.2018)
Published in Scientific reports (21.11.2018)
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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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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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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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