Eur J Clin Invest. 2005 Nov;35(11):711-7.
   Recombinant human erythropoietin: effects on frataxin expression in vitro.

   Sturm B, Stupphann D, Kaun C, Boesch S, Schranzhofer M, Wojta J,
Goldenberg H, Scheiber-Mojdehkar B.

   Medical University of Vienna, General Hospital of Vienna, Vienna,
Austria.

   Background Friedreich's ataxia (FRDA) is a neurodegenerative
disorder caused by decreased expression of the protein frataxin,
recently described to be an iron chaperone for the assembly of
iron-sulphur clusters in the mitochondria, causing iron accumulation
in mitochondria, oxidative stress and cell damage. Searching for
compounds that could possibly influence frataxin expression, we found
that the cytokine recombinant human erythropoietin (rhuEPO)
significantly increases frataxin expression by a still unknown
mechanism. Materials and methods Isolated lymphocytes from FRDA
patients, isolated human cardiac cells (fibroblasts and myocytes) from
patients undergoing heart transplantation and P19 mouse cells
(neuronal typ), were incubated with different concentrations of
rhuEPO, and immunoblot was carried out for the detection of frataxin.
Results We show for the first time that the cytokine recombinant human
erythropoietin (rhuEPO) can, additionally to its reported neuro- and
cardioprotective properties, increase frataxin expression in vitro. We
show that rhuEPO significantly increases frataxin expression in
primary lymphocytes from patients with Friedreich's ataxia. Further we
show that rhuEPO can also increase frataxin expression in many other
cell types; among them the most affected cell types in FRDA such as
neurones and cardiac cells. Conclusions Our results provide a
scientific basis for further studies examining the effectiveness of
this agent for the treatment of FRDA patients. Eur J Clin Invest 2005;
35 (11): 711-717.

   PMID: 16269021 [PubMed - in process]