Article
Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA.
Acta neuropathologica - 1 Oct 2012
Parchi Piero, de Boni Laura, Saverioni Daniela, Cohen Mark L, Ferrer Isidro, Gambetti Pierluigi, Gelpi Ellen, Giaccone Giorgio, Hauw Jean-Jacques, Höftberger Romana, Ironside James W, Jansen Casper, Kovacs Gabor G, Rozemuller Annemieke, Seilhean Danielle, Tagliavini Fabrizio, Giese Armin, Kretzschmar Hans A
Abstract excerpt
The current classification of human sporadic prion diseases recognizes six major phenotypic subtypes with distinctive clinicopathological features, which largely correlate at the molecular level with the genotype at the polymorphic codon 129 (methionine, M, or valine, V) in the prion protein gene and with the size of the protease-resistant core of the abnormal prion protein, PrP(Sc) (i.e. type 1 migrating at 21...
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