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Heat shock factor 1 over-expression protects against exposure of hydrophobic residues on mutant SOD1 and early mortality in a mouse model of amyotrophic lateral sclerosis

BACKGROUND: Mutations in the Cu/Zn superoxide dismutase gene (SOD1) are responsible for 20% of familial forms of amyotrophic lateral sclerosis (ALS), and mutant SOD1 has been shown to have increased surface hydrophobicity in vitro. Mutant SOD1 may adopt a complex array of conformations with varying...

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Detalles Bibliográficos
Autores principales: Lin, Pei-Yi, Simon, Sharotka M, Koh, Won Kyun, Folorunso, Oluwarotimi, Umbaugh, C Samuel, Pierce, Anson
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3907013/
https://www.ncbi.nlm.nih.gov/pubmed/24256636
http://dx.doi.org/10.1186/1750-1326-8-43