Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals

  title={Loss of Frataxin activates the iron/sphingolipid/PDK1/Mef2 pathway in mammals},
  author={Kuchuan Chen and Tammy Szu-Yu Ho and Guang Lin and Kai Li Tan and Matthew N Rasband and Hugo J. Bellen},
Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disease caused by mutations in Frataxin (FXN). Loss of FXN causes impaired mitochondrial function and iron homeostasis. An elevated production of reactive oxygen species (ROS) was previously proposed to contribute to the pathogenesis of FRDA. We recently showed that loss of frataxin homolog (fh), a Drosophila homolog of FXN, causes a ROS independent neurodegeneration in flies (Chen et al., 2016). In fh mutants, iron… CONTINUE READING
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