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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Main Authors: Stevens, Daniel J., Walter, Eric D., Rodríguez, Abel, Draper, David, Davies, Paul, Brown, David R., Millhauser, Glenn L.
Format: Artigo
Jezik:Inglês
Izdano: Public Library of Science 2009
Teme:
Research Article
Online dostop: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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