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Direct and indirect mechanisms for wild-type SOD1 to enhance the toxicity of mutant SOD1 in bigenic transgenic mice

Co-expression of wild-type human superoxide dismutase 1 (WT-hSOD1) with ALS mutant hSOD1 accelerates disease onset relative to mice expressing only mutant protein. Here, we analyzed the effect of co-expressed WT-hSOD1 in two established mutant mouse models (L126Z and G37R), and a new model that expr...

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Detalhes bibliográficos
Publicado no:Hum Mol Genet
Main Authors: Xu, Guilian, Ayers, Jacob I., Roberts, Brittany L., Brown, Hilda, Fromholt, Susan, Green, Cameron, Borchelt, David R.
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2015
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4375410/
https://ncbi.nlm.nih.gov/pubmed/25305079
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddu517
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