Article
Novel strain properties distinguishing sporadic prion diseases sharing prion protein genotype and prion type.
Scientific reports - 16 Jan 2017
Cracco Laura, Notari Silvio, Cali Ignazio, Sy Man-Sun, Chen Shu G, Cohen Mark L, Ghetti Bernardino, Appleby Brian S, Zou Wen-Quan, Caughey Byron, Safar Jiri G, Gambetti Pierluigi
Abstract excerpt
In most human sporadic prion diseases the phenotype is consistently associated with specific pairings of the genotype at codon 129 of the prion protein gene and conformational properties of the scrapie PrP (PrPSc) grossly identified types 1 and 2. This association suggests that the 129 genotype favours the selection of a distinct strain that in turn determines the phenotype. However, this mechanism cannot play a...
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