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A team of researchers …have found a key link between prions and the neurodegenerative disease amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease.
…These findings provide a molecular explanation for the progressive spread of ALS through the nervous system, and highlight the central role of the propagation of misfolded proteins in the pathogenesis of neurodegenerative diseases, including ALS, Alzheimer's and Parkinson's.
Key Finding: "Intermolecular transmission of SOD-1 misfolding in living cells" - Published in the Proceedings of the National Academy of Sciences (PNAS), September 2011
* The paper shows that superoxide dismutase 1 (SOD1) participates in template-directed misfolding, in other words, the coercion of one protein by another protein to change shape and aggregate such as prion diseases do.
* The results will be significant to the ALS field because it connects prion mechanisms behind the biological progression of ALS, and provides a molecular explanation for the linear and temporal spread of ALS through the nervous system.
* Furthermore, the research has identified a specific molecular target, which when manipulated, halts the conversion of SOD1 to a misfolded, disease-causing form. This is a first-step towards the development of targeted treatments that may stop ALS, which PrioNet is further exploiting through newly-funded research.
* This research was supported by PrioNet Canada and in part by Amorfix Life Sciences and the Canadian Institutes of Health Research.
Originally posted by soficrow
In ALS - amyotrophic lateral sclerosis, also known as Lou Gehrig's disease - a protein misfolds, spreads through the nervous system and causes progressive neurodegeneration.