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Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models

Mutants of superoxide dismutase-1 (SOD1) cause ALS by an unidentified cytotoxic mechanism. We have previously shown that the stable SOD1 mutants D90A and G93A are abundant and show the highest levels in liver and kidney in transgenic murine ALS models, whereas the unstable G85R and G127X mutants are...

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Autors principals: Zetterström, Per, Stewart, Heather G., Bergemalm, Daniel, Jonsson, P. Andreas, Graffmo, Karin S., Andersen, Peter M., Brännström, Thomas, Oliveberg, Mikael, Marklund, Stefan L.
Format: Artigo
Idioma:Inglês
Publicat: National Academy of Sciences 2007
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC1955813/
https://ncbi.nlm.nih.gov/pubmed/17715066
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0700477104
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