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Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein.

Wen-Quan Zou, Gianfranco Puoti, Xiangzhu Xiao, Jue Yuan, Liuting Qing, Ignazio Cali, Miyuki Shimoji, Jan P M Langeveld, Rudy Castellani, Silvio Notari, Barbara Crain, Robert E Schmidt, Michael Geschwind, Stephen J Dearmond, Nigel J Cairns, Dennis Dickson, Lawrence Honig, Juan Maria Torres, James Mastrianni, Sabina Capellari, Giorgio Giaccone, Ermias D Belay, Lawrence B Schonberger, Mark Cohen, George Perry, Qingzhong Kong, Piero Parchi, Fabrizio Tagliavini, and Pierluigi Gambetti

Ann Neurol 68(2):162-72 (2010) PMID 20695009 PMCID PMC3032610

The objective of the study is to report 2 new genotypic forms of protease-sensitive prionopathy (PSPr), a novel prion disease described in 2008, in 11 subjects all homozygous for valine at codon 129 of the prion protein (PrP) gene (129VV). The 2 new PSPr forms affect individuals who are either homozygous for methionine (129MM) or heterozygous for methionine/valine (129MV).

DOI: 10.1002/ana.22094
Version: za2963e q8za2 q8zb8 q8zc9 q8zd7 q8zec q8zfa q8zge
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