Protein aggregation in motor neurone disorders.

  title={Protein aggregation in motor neurone disorders.},
  author={Jon D Wood and Tim Beaujeux and Pamela J Shaw},
  journal={Neuropathology and applied neurobiology},
  volume={29 6},
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


Publications citing this paper.
Showing 1-10 of 53 extracted citations

Similar Papers

Loading similar papers…