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Protein aggregation in motor neurone disorders

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.