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Motor neuron disease

Mutant SOD1 induces misfolding of the wild-type protein

Nature Reviews Neurology volume 7, page 597 (2011)Cite this article

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Over 150 mutations in the superoxide dismutase 1 (SOD1) gene have been associated with amyotrophic lateral sclerosis (ALS), but the disease-causing mechanisms are still being elucidated. Previous studies demonstrated SOD1 aggregates in motor neurons from patients with SOD1-ALS, and new research shows that misfolded mutant SOD1 protein can induce misfolding of the wild-type protein, thereby enabling propagation of SOD1 aggregates via a prion-like mechanism.

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ORIGINAL RESEARCH PAPER

  1. Grad, L. I. et al. Intermolecular transmission of superoxide dismutase 1 misfolding in living cells. Proc. Natl Acad. Sci. USA 108, 16398–16403 (2011)

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Mutant SOD1 induces misfolding of the wild-type protein. Nat Rev Neurol 7, 597 (2011). https://doi.org/10.1038/nrneurol.2011.171

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