Mutant SOD1-expressing astrocytes release toxic factors that trigger motoneuron death by inducing hyperexcitability.

@article{Fritz2013MutantSA,
  title={Mutant SOD1-expressing astrocytes release toxic factors that trigger motoneuron death by inducing hyperexcitability.},
  author={Elsa Fritz and Pamela Izaurieta and Alexandra Dabreo Weiss and Franco R. Mir and Patricio Gajardo Rojas and Dioselin Gonzalez and Fabiola Rojas and R. H. Brown and Rodolfo Madrid and Brigitte van Zundert},
  journal={Journal of neurophysiology},
  year={2013},
  volume={109 11},
  pages={2803-14}
}
Amyotrophic lateral sclerosis (ALS) is a devastating paralytic disorder caused by dysfunction and degeneration of motoneurons starting in adulthood. Recent studies using cell or animal models document that astrocytes expressing disease-causing mutations of human superoxide dismutase 1 (hSOD1) contribute to the pathogenesis of ALS by releasing a neurotoxic factor(s). Neither the mechanism by which this neurotoxic factor induces motoneuron death nor its cellular site of action has been elucidated… CONTINUE READING
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