Article
Protein aggregation and protein instability govern familial amyotrophic lateral sclerosis patient survival.
PLoS biology - 29 Jul 2008
Wang Qi, Johnson Joshua L, Agar Nathalie Y R, Agar Jeffrey N
Abstract excerpt
The nature of the "toxic gain of function" that results from amyotrophic lateral sclerosis (ALS)-, Parkinson-, and Alzheimer-related mutations is a matter of debate. As a result no adequate model of any neurodegenerative disease etiology exists. We demonstrate that two synergistic properties, namely, increased protein aggregation propensity (increased likelihood that an unfolded protein will aggregate) and...
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