Article
Prion protein repeat expansion results in increased aggregation and reveals phenotypic variability.
Molecular and cellular biology - 1 Aug 2007
Tank Elizabeth M H, Harris David A, Desai Amar A, True Heather L
Abstract excerpt
Mammalian prion diseases are fatal neurodegenerative disorders dependent on the prion protein PrP. Expansion of the oligopeptide repeats (ORE) found in PrP is associated with inherited prion diseases. Patients with ORE frequently harbor PrP aggregates, but other factors may contribute to pathology, as they often present with unexplained phenotypic variability. We created chimeric yeast-mammalian prion proteins to...
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