Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models.

@article{Zetterstrm2007SolubleMS,
  title={Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models.},
  author={Per Zetterstr{\"o}m and Heather G Stewart and Daniel Bergemalm and Per Andreas Jonsson and Karin Sixtensdotter Graffmo and Peter Munch Andersen and Thomas Br{\"a}nnstr{\"o}m and Mikael Oliveberg and Stefan L Marklund},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2007},
  volume={104 35},
  pages={14157-62}
}
Mutants of superoxide dismutase-1 (SOD1) cause ALS by an unidentified cytotoxic mechanism. We have previously shown that the stable SOD1 mutants D90A and G93A are abundant and show the highest levels in liver and kidney in transgenic murine ALS models, whereas the unstable G85R and G127X mutants are scarce but enriched in the CNS. These data indicated that minute amounts of misfolded SOD1 enriched in the motor areas might exert the ALS-causing cytotoxicity. A hydrophobic interaction… CONTINUE READING

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