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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...
Autores principales: | , , , , , , |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2009
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2663819/ https://www.ncbi.nlm.nih.gov/pubmed/19381258 http://dx.doi.org/10.1371/journal.ppat.1000390 |