Antiprogesterone therapy uncouples axonal loss from demyelination in a transgenic rat model of CMT1A neuropathy.

@article{Horste2007AntiprogesteroneTU,
  title={Antiprogesterone therapy uncouples axonal loss from demyelination in a transgenic rat model of CMT1A neuropathy.},
  author={Gerd Meyer Zu Horste and Thomas Prukop and David Liebetanz and W. Mobius and Klaus-Armin Nave and Michael Werner Sereda},
  journal={Annals of neurology},
  year={2007},
  volume={61 1},
  pages={61-72}
}
OBJECTIVE Charcot-Marie-Tooth disease (CMT) is the most common inherited neuropathy, and a duplication of the Pmp22 gene causes the most frequent subform CMT1A. Using a transgenic rat model of CMT1A, we tested the hypothesis that long-term treatment with anti-progesterone (Onapristone) reduces Pmp22 overexpression and improves CMT disease phenotype of older animals, thereby extending a previous proof-of-concept observation in a more clinically relevant setting. METHODS We applied placebo… CONTINUE READING

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