RESEARCH GRANT AWARD SUMMARIES
The following received research grant funding from the National Ataxia Foundation for the fiscal year of 2008. A summary of the completed research studies will be published in Generations in 2009:
- Role of rhu-EPO and HDAC inhibitors alone and/or in combination on the regulation of frataxin expression. New prospective for FRDA treatment, Sergio Cocozza, MD
- Frataxin folding, chaperone role and interactions with iron-sulfur biosynthesis proteins: contributions to understand Friedreich's ataxia, Cláudio M. Gomes, PhD
- Disrupted pre-mRNA splicing in Machado-Joseph disease, Michael D. Hebert, PhD
- Molecular and Cellular Physiology of Episodic Ataxia Type 1, EA1, James Maylie, PhD
- Dissecting transcriptional misregulation in SCA1 using Laser capture microscopy and transcriptional profiling, Puneet Opal, MD, PhD
- Biological tools to investigate Spinocerebellar Ataxia 27 (SCA27), David M. Ornitz, M.D.,Ph.D.
- RNAi as therapy for spinocerebellar ataxia type 3 (SCA3), Henry Paulson, MD, PhD
- Role of Bergmann glia in Purkinje cell development and pathology in SCA1, Parminder J. S. Vig, Ph.D.
- RNAi therapeutics for Friedreich's ataxia, Robert Wilson, MD, PhD
- Understanding the molecular basis of rCGG mediated neurodegeneration in Drosophila melanogaster, Qurashi, Abrar, PhD
- P130Cas in granule cell migration: understanding the mechanisms underlying cell migration in cerebellar development, Riccomagno, Martin M., PhD
- SCA 2 Pathogenesis: Altered splicing caused by gain of normal ataxin-2 function, Strauss, Karsten, PhD
- Elucidating the mechanism of ATX-2: the C.elegans ortholog of a protein implicated in human neurodegenerative disease, Wright, Jane, PhD
- Pathogenesis of impaired glucose tolerance and diabetes in Friedreich's ataxia: contribution of insulin resistance and pancreatic beta cell dysfunction, Miriam Cnop, MD, PhD
- The role of frataxin in heme-synthesis, Dr. Brigitte Sturm
- Use of a novel catalytic antioxidant, CTMIO, in a GAA repeat mouse model of FRDA Project Objectives, Nuri Gueven, PhD, Senior Research Officer
- Development of Methods to Determine the Pharmacodynamic Effect of HDAC Inhibitors on Frataxin Expression, James R. Rusche, PhD
- Preclinical Markers of Cardiomyopathy in Friedreich's ataxia, Subha V. Raman, M.D., and Roula al-Dahhak, M.D.
