0000000000076769
AUTHOR
José A. Botella
Altered lipid metabolism in a Drosophila model of Friedreich's ataxia
13 páginas, 5 figuras.-- et al.
Overexpression of Human and Fly Frataxins in Drosophila Provokes Deleterious Effects at Biochemical, Physiological and Developmental Levels
10 pages, 5 figures. 21779322[PubMed] PMCID: PMC3136927
Causative role of oxidative stress in a Drosophila model of Friedreich ataxia
Friedreich ataxia (FA), the most common form of hereditary ataxia, is caused by a deficit in the mitochondrial protein frataxin. While several hypotheses have been suggested, frataxin function is not well understood. Oxidative stress has been suggested to play a role in the pathophysiology of FA, but this view has been recently questioned, and its link to frataxin is unclear. Here, we report the use of RNA interference (RNAi) to suppress the Drosophila frataxin gene (fh) expression. This model system parallels the situation in FA patients, namely a moderate systemic reduction of frataxin levels compatible with normal embryonic development. Under these conditions, fh-RNAi flies showed a shor…