Article
Novel prion protein conformation and glycotype in Creutzfeldt-Jakob disease.
Archives of neurology - 1 Apr 2007
Zanusso Gianluigi, Polo Alberto, Farinazzo Alessia, Nonno Romolo, Cardone Franco, Di Bari Michele, Ferrari Sergio, Principe Serena, Gelati Matteo, Fasoli Elisa, Fiorini Michele, Prelli Frances, Frangione Blas, Tridente Giuseppe, Bentivoglio Marina, Giorgi Alessandra, Schininà Maria Eugenia, Maras Bruno, Agrimi Umberto, Rizzuto Nicola, Pocchiari Maurizio, Monaco Salvatore
Abstract excerpt
OBJECTIVE: To describe a novel molecular and pathological phenotype of Creutzfeldt-Jakob disease. Patient A 69-year-old woman with behavioral and personality changes followed by rapidly evolving dementia. RESULTS: Postmortem examination of the brain showed intracellular prion protein deposition and axonal swellings filled with amyloid fibrils. Biochemical analysis of the pathological prion protein disclosed a...
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