Protein aggregation in motor neurone disorders.

@article{Wood2003ProteinAI,
  title={Protein aggregation in motor neurone disorders.},
  author={Jon D Wood and Tim Beaujeux and Pamela J Shaw},
  journal={Neuropathology and applied neurobiology},
  year={2003},
  volume={29 6},
  pages={529-45}
}
Toxicity associated with abnormal protein folding and protein aggregation are major hypotheses for neurodegeneration. This article comparatively reviews the experimental and human tissue-based evidence for the involvement of such mechanisms in neuronal death associated with the motor system disorders of X-linked spinobulbar muscular atrophy (SBMA; Kennedy's disease) and amyotrophic lateral sclerosis (ALS), especially disease related to mutations in the superoxide dismutase (SOD1) gene. Evidence… CONTINUE READING

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