Metal-deficient SOD1 in amyotrophic lateral sclerosis

@article{Hilton2015MetaldeficientSI,
  title={Metal-deficient SOD1 in amyotrophic lateral sclerosis},
  author={J. B. Hilton and A. White and P. Crouch},
  journal={Journal of Molecular Medicine (Berlin, Germany)},
  year={2015},
  volume={93},
  pages={481 - 487}
}
  • J. B. Hilton, A. White, P. Crouch
  • Published 2015
  • Chemistry, Medicine
  • Journal of Molecular Medicine (Berlin, Germany)
  • Mutations to the ubiquitous antioxidant enzyme Cu/Zn superoxide dismutase (SOD1) were the first established genetic cause of the fatal, adult-onset neurodegenerative disease amyotrophic lateral sclerosis (ALS). It is widely accepted that these mutations do not cause ALS via a loss of antioxidant function, but elucidating the alternate toxic gain of function has proven to be elusive. Under physiological conditions, SOD1 binds one copper ion and one zinc ion per monomer to form a highly stable… CONTINUE READING
    37 Citations

    Figures and Topics from this paper

    New links between SOD1 and metabolic dysfunction from a yeast model of amyotrophic lateral sclerosis
    • 33
    • PDF
    Anatomical redistribution of endogenous copper in embryonic mice overexpressing SOD1.
    • 5
    Ionic Homeostasis Maintenance in ALS: Focus on New Therapeutic Targets
    • 19
    • PDF

    References

    SHOWING 1-10 OF 78 REFERENCES
    A role for copper in the toxicity of zinc-deficient superoxide dismutase to motor neurons in amyotrophic lateral sclerosis.
    • 63
    Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis.
    • 463
    • PDF
    ALS-causing SOD1 mutations promote production of copper-deficient misfolded species.
    • 42
    Insoluble Mutant SOD1 Is Partly Oligoubiquitinated in Amyotrophic Lateral Sclerosis Mice*
    • 98
    • PDF
    Initiation and elongation in fibrillation of ALS-linked superoxide dismutase
    • 193
    • PDF
    Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis
    • 6,106