Aggregation propensities of superoxide dismutase G93 hotspot mutants mirror ALS clinical phenotypes

Protein framework alterations in heritable Cu, Zn superoxide dismutase (SOD) mutants cause misassembly and aggregation in cells affected by the motor neuron disease ALS. However, the mechanistic relationship between superoxide dismutase 1 (SOD1) mutations and human disease is controversial, with man...

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Autori principali: Pratt, Ashley J., Shin, David S., Merz, Gregory E., Rambo, Robert P., Lancaster, W. Andrew, Dyer, Kevin N., Borbat, Peter P., Poole, Farris L., Adams, Michael W. W., Freed, Jack H., Crane, Brian R., Tainer, John A., Getzoff, Elizabeth D.
Natura: Artigo
Lingua:Inglês
Pubblicazione: National Academy of Sciences 2014
Soggetti:
PNAS Plus
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4217430/
https://ncbi.nlm.nih.gov/pubmed/25316790
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1308531111
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