The copper chelator d-penicillamine delays onset of disease and extends survival in a transgenic mouse model of familial amyotrophic lateral sclerosis.

@article{Hottinger1997TheCC,
  title={The copper chelator d-penicillamine delays onset of disease and extends survival in a transgenic mouse model of familial amyotrophic lateral sclerosis.},
  author={Andreas F. Hottinger and Eric G Fine and Mark E Gurney and Anne D. Zurn and P. Aebischer},
  journal={The European journal of neuroscience},
  year={1997},
  volume={9 7},
  pages={1548-51}
}
A subpopulation of familial cases of amyotrophic lateral sclerosis has been linked to mutations in the gene encoding Cu/Zn superoxide dismutase (SOD1). There is in vitro evidence that certain SOD1 mutants, in addition to their normal dismutation function, show increased ability of the enzyme to act as a peroxidase. This reaction is sensitive to inhibition by copper chelators. To test this hypothesis in vivo, we administered the copper chelator d-penicillamine to a transgenic mouse model of… CONTINUE READING