From charcot to lou gehrig: deciphering selective motor neuron death in als

@article{Cleveland2001FromCT,
  title={From charcot to lou gehrig: deciphering selective motor neuron death in als},
  author={D. Cleveland and J. Rothstein},
  journal={Nature Reviews Neuroscience},
  year={2001},
  volume={2},
  pages={806-819}
}
Since its description by Charcot more than 130 years ago, the mechanism underlying the characteristic selective degeneration and death of motor neurons in amyotrophic lateral sclerosis has remained a mystery. Modern genetics has now identified mutations in two genes — SOD1 and ALS2 — as primary causes of the disease, and has implicated others as potential contributors. Insights into these abnormalities, together with errors in the handling of synaptic glutamate and the potential excitotoxic… Expand
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