Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

Many mutations confer upon copper/zinc superoxide dismutase-1 (SOD1) one or more toxic function(s) that impair motor neuron viability and cause familial amyotrophic lateral sclerosis (FALS). Using a conformation-specific antibody that detects misfolded SOD1 (C4F6), we demonstrate that oxidized WT-SO...

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Detalles Bibliográficos
Autores principales: Bosco, Daryl A., Morfini, Gerardo, Karabacak, N. Murat, Song, Yuyu, Gros-Louis, Francois, Pasinelli, Piera, Goolsby, Holly, Fontaine, Benjamin A., Lemay, Nathan, McKenna-Yasek, Diane, Frosch, Matthew P., Agar, Jeffery N., Julien, Jean-Pierre, Brady, Scott T., Brown, Robert H.
Formato: Texto
Lenguaje:English
Publicado: 2010
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2967729/
https://www.ncbi.nlm.nih.gov/pubmed/20953194
http://dx.doi.org/10.1038/nn.2660

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