Les ataxies cérébelleuses autosomiques récessives

Original article in French, abstract in English Autosomal recessive cerebellar ataxias

Revue Neurologique, Article in Press, Corrected Proof
doi:10.1016/j.neurol.2010.07.021

M. Anheim, Service de neurogénétique, hôpital de la Pitié-Salpêtrière, Paris, France, Centre de référence des maladies neurogénétiques de l’enfant et de l’adulte, Paris, France, Unité Inserm UMR_S975 CRICM, hôpital de la Pitié-Salpêtrière, Paris, France

Keywords: Ataxia; Cerebellum; Autosomal recessive; Oculomotor apraxia; Peripheral neuropathy; Friedreich's ataxia

METHODS OF IDENTIFYING HISTONE DEACETYLASE INHIBITORS USEFUL FOR NEUROLOGICAL DISORDERS

United States Patent Application 20100285476

Inventors:Rusche, James R. (Senior Vice President of Research & Development, Repligen Corporation)
Cooper, Andrew (Senior Research Scientist at Repligen Corporation)

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Activation of mitochondrial energy metabolism protects against cardiac failure.

Aging (Albany NY). 2010 Nov 16.

Schulz TJ, Westermann D, Isken F, Voigt A, Laube B, Thierbach R, Kuhlow D, Zurse K, Schomburg L, Pfeiffer AF, Tschöpe C, Ristow M.
Department of Human Nutrition, Institute of Nutrition, University of Jena, Germany.

Keywords: Cardiac failure, impaired energy homeostasis in the heart, Mitochondrial metabolism, over-expressing the mitochondrial protein frataxin, iron-sulfur-cluster biogenesis, mitochondrial electron flux, stress defense mechanisms, cardiomyopathy, insulin/IGF-1 signaling cascade, protein kinase B, Akt, glycogen synthase kinase 3.

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,