Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells.

  title={Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells.},
  author={Christian M{\"u}nch and John O'Brien and Anne Bertolotti},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  volume={108 9},
Deposition of proteins of aberrant conformation is the hallmark of many neurodegenerative diseases. Misfolding of the normally globular mutant superoxide dismutase-1 (SOD1) is a central, early, but poorly understood event in the pathogenic cascade leading to familial forms of ALS. Here we report that aggregates composed of an ALS-causing SOD1 mutant penetrate inside cells by macropinocytosis and rapidly exit the macropinocytic compartment to nucleate aggregation of the cytosolic, otherwise… CONTINUE READING
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