Erythropoietin and small molecule agonists of the tissue-protective erythropoietin receptor increase FXN expression in neuronal cells in vitro and in Fxn-deficient KIKO mice in vivo

@article{Miller2017ErythropoietinAS,
  title={Erythropoietin and small molecule agonists of the tissue-protective erythropoietin receptor increase FXN expression in neuronal cells in vitro and in Fxn-deficient KIKO mice in vivo
},
  author={James L. Miller and Myriam Rai and N. Frigon and M. Pandolfo and J. Punnonen and J. Spencer},
  journal={Neuropharmacology},
  year={2017},
  volume={123},
  pages={34-45}
}
&NA; Friedreich's ataxia (FA) is a progressive neurodegenerative disease caused by reduced levels of the mitochondrial protein frataxin (FXN). Recombinant human erythropoietin (rhEPO) increased FXN protein in vitro and in early clinical studies, while no published reports evaluate rhEPO in animal models of FA. STS‐E412 and STS‐E424 are novel small molecule agonists of the tissue‐protective, but not the erythropoietic EPO receptor. We find that rhEPO, STS‐E412 and STS‐E424 increase FXN… Expand
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