The Test of Everyday Attention Reveals Significant Sustained Volitional Attention and Working Memory Deficits in Friedreich Ataxia.

J Int Neuropsychol Soc. 2010 Nov 17:1-5. [Epub ahead of print]

Klopper F, Delatycki MB, Corben LA, Bradshaw JL, Rance G, Georgiou-Karistianis N.
Experimental Neuropsychology Research Unit, School of Psychology and Psychiatry, Monash University, Clayton, Victoria, Australia.

Keywords: Sustained volitional attention, working memory capacity, Friedreich ataxia (FRDA), Test of Everyday Attention, IQ-matched controls, GAA repeat number, FXN gene, disease severity, corticocerebellar pathways, cortical and/or cerebellar pathology.

Development and assessment of selective mitochondrion-targeted iron chelators for the study of the neurological disorder Friedrich’s Ataxia

Department of Pharmacy & Pharmacology, University of Bath

NEW PROJECT

"we will investigate the conjugation of a variety of clinical strong iron chelator molecules to cell-penetrating and mitochondria-targeting peptide sequences, to create highly specific cell-permeable metal chelators"

Development of Biomarkers for HDAC Inhibitor Treatment of Friedreich's Ataxia

AAN 2010 - American Academy of Neurology

Massimo Pandolfo, Brussels, Belgium, Heather Plasterer, Waltham, MA, Myriam Rai, Brussels, Belgium, Sushil Sharma, Carly Therkelsen, Andrew Cooper, Waltham, MA, Jennifer Farmer, David Lynch, Philadelphia, PA, James Rusche, Waltham, MA

Effects of Erythropoietin on Endothelial Progenitor Cells, Frataxin Levels and Oxidative Metabolism in the Skeletal Muscle of Friedreich Ataxia Patients

AAN 2010 - American Academy of Neurology?

Wolfgang Nachbauer, Julia Wanschitz, Markus Reindl, Innsbruck, Austria, Barbara Scheiber-Mojdehkar, Vienna, Austria, Werner Poewe, Sylvia Boesch, Innsbruck, Austria

STATegics, Inc. Announces Award from the Department of Defense to Advance Proprietary Small Molecule Mimetics of Erythropoietin for the Treatment of Traumatic Brain Injury

SUNNYVALE, Calif., Nov. 16, 2010 (GLOBE NEWSWIRE)
STATegics' programs have been supported by grants from the National Institute of Neurological Disorders and Stroke (NINDS), Friedreich's Ataxia Research Alliance (FARA) and the U.S. Government's Qualifying Therapeutic Discovery Project (QTDP) program.

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The lead indication for the erythropoietin mimetics is Friedreich's ataxia,
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Whole-body vibration alters blood flow velocity and neuromuscular activity in Friedreich’s ataxia

Clinical Physiology and Functional Imaging, no. doi: 10.1111/j.1475-097X.2010.00992.x
Herrero, A. J., Martín, J., Martín, T., García-López, D., Garatachea, N., Jiménez, B. and Marín, P. J.

Keywords: Doppler ultrasound, electromyography, oscillating platform, rate of perceived exertion, rehabilitation, vibration frequency.

Re-Engineering Erythropoietin as an IgG Fusion Protein That Penetrates the Blood−Brain Barrier in the Mouse

Mol. Pharmaceutics, Article ASAP, DOI: 10.1021/mp1001763

 

Qing-Hui Zhou^†, Ruben J. Boado^†‡, Jeff Zhiqiang Lu^‡, Eric Ka-Wai Hui^‡, and William M. Pardridge*^†

Department of Medicine, UCLA, Los Angeles, California 90024, United States, and ArmaGen Technologies, Inc., Santa Monica, California 90401, United States

 

Keywords: drug targeting; drug delivery; Erythropoietin (EPO),  neuroprotective agent,  blood−brain barrier (BBB), chimeric monoclonal antibody (mAb),  transferrin receptor (TfR),  cTfRMAb-EPO, fusion protein,  IgG fusion protein,

 

DockAnalyse: an application for the analysis of protein-protein interactions

BMC Structural Biology 2010, 10:37doi:10.1186/1472-6807-10-37

Isaac Amela , Pedro Delicado , Antonio Gomez , Silvia Bonas , Enrique Querol and Juan Cedano

Institut de Biotecnologia i de Biomedicina Parc de Recerca UAB and Departament de Bioquímica i Biologia Molecular, Universitat Autònoma de Barcelona. 08193-Bellaterra, Barcelona, Spain
Departament d’Estadística i Investigació Operativa, Universitat Politècnica de Catalunya. 08034-Barcelona, Spain.

OPEN ACCESS

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Most of the studies prompt the suggestion that the iron and sulfur atoms required for the ISC biogenesis on Isu1/Isu2 are donated by other proteins, named Frataxin and Nfs1 [20]. This ISC biogenesis machinery is not yet well understood and problems in it cause several human diseases linked to protein/enzyme deficits.

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Iron-Sulfur Clusters: Biogenesis, Molecular Mechanisms and Their Functional Significance

Iron-Sulfur Clusters: Biogenesis, Molecular Mechanisms and Their Functional Significance

Xiang Ming Xu, Simon Geir Møller. Antioxidants & Redox Signaling. -Not available-, ahead of print. doi:10.1089/ars.2010.3259.

Keywords:Iron-sulfur clusters [Fe-S], inorganic cofactors, regulation of enzyme activity, mitochondrial respiration, ribosome biogenesis, cofactor biogenesis, gene expression regulation, nucleotide metabolism, mitochondria, human diseases.

Spectral measures of the effects of Friedreich's ataxia on speech.

Int J Speech Lang Pathol. 2010 Nov 9. [Epub ahead of print]

Rosen KM, Folker JE, Murdoch BE, Vogel AP, Cahill LM, Delatycki MB, Corben LA.
The University of Queensland, Brisbane, Australia.

Keywords:Friedreich's ataxia (FRDA), speech motor control, dysarthria, ,speaking task, spectral variation.