Sunday, August 21, 2016

Cerebral and cerebellar grey matter atrophy in Friedreich ataxia: the IMAGE-FRDA study.

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Selvadurai LP , Harding IH , Corben LA , Stagnitti MR , Storey E , Egan GF , Delatycki MB , Georgiou-Karistianis N J Neurol [2016] doi:10.1...
Saturday, August 20, 2016

Increased Frataxin Levels Protect Retinal Ganglion Cells After Acute Ischemia/Reperfusion in the Mouse Retina In Vivo

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Rowena Schultz; Otto W. Witte; Christian Schmeer; Investigative Ophthalmology & Visual Science August 2016, Vol.57, 4115-4124. doi:10.11...
Friday, August 19, 2016

Lentivirus-meditated frataxin gene delivery reverses genome instability in Friedreich ataxia patient and mouse model fibroblasts

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Khonsari H , Schneider M , Al-Mahdawi S , Chianea YG , Themis M , Parris C , Pook MA , Themis M; Gene Therapy accepted article preview 12 Au...
Wednesday, August 17, 2016

Frataxin silencing alters microtubule stability in motor neurons: implications for Friedreich's Ataxia

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Emanuela Piermarini, Daniele Cartelli, Anna Pastore, Giulia Tozzi, Claudia Compagnucci, Ezio Giorda, Jessica D’Amico, Stefania Petrini, Enri...
Sunday, August 14, 2016

Architecture of the Human Mitochondrial Iron-Sulfur Cluster Assembly Machinery

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Oleksandr Gakh, Wasantha Ranatunga, Douglas Y. Smith IV, Eva-Christina Ahlgren, Salam Al-Karadaghi, James R. Thompson and Grazia Isaya. JBC,...
Thursday, August 11, 2016

Characterization of Novel Small-Molecule NRF2 Activators: Structural and Biochemical Validation of Stereospecific KEAP1 Binding

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Carlos Huerta, Xin Jiang, Isaac Trevino, Christopher F. Bender, Deborah A. Ferguson, Brandon Probst, Kerren K. Swinger, Vincent S. Stoll, Ph...
Wednesday, August 10, 2016

Synthetic Nucleic Acids and Treatment of Neurological Diseases

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David R. Corey, PhD, JAMA Neurol. Published online August 01, 2016. doi:10.1001/jamaneurol.2016.2089 OPEN EMERGING TARGET: FRATAXIN/...
Tuesday, August 9, 2016

Variable sensory nerve conduction parameters in late onset Friedreich ataxia

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Alix, J. J.P., Alam, T., Garrard, K., Martindale, J., Shanmugarajah, P., Rao, D. G. and Hadjivassiliou, M. (2016). Muscle Nerve. Accepted Au...
Monday, August 8, 2016

Long-Axis Left Ventricular and Left Atrial Dysfunction in Friedreich Ataxia with Normal Ejection Fraction – Global Longitudinal Strain Versus Tissue Doppler Imaging Velocities

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D. Jackson, R. Hassam, L. Donelan, R. Peverill, Heart, Lung and Circulation, Volume 25, Supplement 2, August 2016, Page S80, ISSN 1443-9506,...
Sunday, August 7, 2016

Translating HDAC inhibitors in Friedreich’s ataxia

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Elisabetta Soragni & Joel M. Gottesfeld. Expert Opinion on Orphan Drugs, Published online: 31 Jul 2016 DOI:10.1080/21678707.2016.1215910...