Effect of copper and disulfiram combination therapy on the macular mouse, a model of Menkes disease.

@article{Bhadhprasit2012EffectOC,
  title={Effect of copper and disulfiram combination therapy on the macular mouse, a model of Menkes disease.},
  author={Wattanaporn Bhadhprasit and Hiroko Kodama and Chie Fujisawa and Tomoko Hiroki and Eishin Ogawa},
  journal={Journal of trace elements in medicine and biology : organ of the Society for Minerals and Trace Elements},
  year={2012},
  volume={26 2-3},
  pages={105-8}
}
Menkes disease (MD) is a genetic neurodegenerative disorder characterized by copper deficiency due to a defect in ATP7A. Standard treatment involves parenteral copper-histidine administration. However, the treatment is ineffective if initiated after two months of age, because the administered copper accumulates in the blood-brain barrier and is not transported to neurons. To resolve this issue, we investigated the effects of a combination therapy comprising copper and disulfiram, a lipophilic… CONTINUE READING
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