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Time-resolved functional analysis of acute impairment of frataxin expression in an inducible cell model of Friedreich ataxia

This group has developed a new model of Friedreich's Ataxia, in which they can switch on or off the loss of frataxin in mouse embryonic fibroblasts . After loss of frataxin protein, cell division, aconitase activity and oxygen consumption rates were decreased, while ROS production was increased. A time-resolved analysis revealed the loss of aconitase activity as an initial event after induction of complete frataxin deficiency, followed by secondarily elevated ROS production and a late increase in iron content. Initial impairments of oxygen consumption and ATP production were found to be compensated in the late state and seemed to play a minor role in Friedreich ataxia pathophysiology. In conclusion and as predicted from its proposed role in iron sulfur cluster (ISC) biosynthesis, disruption of frataxin primarily causes impaired function of ISC-containing enzymes, whereas other consequences, including elevated ROS production and iron accumulation, appear secondary. These parameters and the robustness of the newly established system may additionally be used for a time-resolved study of pharmacological candidates in a high throughput manner.

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Jen Farmer

Jen Farmer

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