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Early Onset Prion Disease from Octarepeat Expansion Correlates with Copper Binding Properties

Insertional mutations leading to expansion of the octarepeat domain of the prion protein (PrP) are directly linked to prion disease. While normal PrP has four PHGGGWGQ octapeptide segments in its flexible N-terminal domain, expanded forms may have up to nine additional octapeptide inserts. The type...

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Detalhes bibliográficos
Main Authors: Stevens, Daniel J., Walter, Eric D., Rodríguez, Abel, Draper, David, Davies, Paul, Brown, David R., Millhauser, Glenn L.
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2009
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2663819/
https://ncbi.nlm.nih.gov/pubmed/19381258
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.ppat.1000390
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