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Research Funded
Contributions to this event go directly to particular research projects, not into a general fund that is then tapped.
These two California grants, totaling about $42,000, were predominantly funded by the proceeds of the August, 2004 event. They were awarded in August or September of 2004.
First Grant, $12,750
Principal Investigator: Giovanni Coppola, UCLA
Dr. Coppola is from Italy (where he worked with Dr. Filla) and he is working now with Dr. Geschwind at UCLA. They are collaborating with Dr. Arnie Koeppen and Dr. Michael Murphy, among others.His project is three fold:
- To Study Cardiac and Skeletal Muscle in a Mouse Model of Frataxin Deficiency, so as to Further Our Understanding of Disease Pathogenesis.
- Confirmation of Gene Expression Changes in Mouse and Human Brain.
- Treatment Alterations in Gene Expression.
A frataxin-deficient mouse model, in early steps of disease pathogenesis and with definite alterations in gene expression, may be a powerful tool in evaluating treatment strategies.
The gene expression patterns in mice treated with antioxidant drugs will be compared to those in non-treated frataxin-deficient mice. The study will involve a compound studied (idebenone) and one not yet studied in vivo (a mitochondria-targeted antioxidant).
Second Grant, $29,930
Principal Investigator: Dr. Michael Baudry, USC.
The title of his grant is Potential Therapeutic Value of "Mito-Protective" Superoxide Dismutase/Catalase Mimetics for Friedreich's Ataxia. His goal is to investigate the possibility of using SOD/catalase mimetics as therapeutics for Friedreich Ataxia.
He has some compounds that he feels are very promising and he now has the mouse model on which to try these compounds. Dr. Baudry is from France and has been in the states since the late 70's. In his application he states, "Overall, these data suggest that............ (these compounds) are most effective as brain "mito-protective" agents and, therefore, very good candidates for evaluation as potential FRDA treatments."
