Allelic Origin of Protease-Sensitive and Protease-Resistant Prion Protein Isoforms in Gerstmann-Sträussler-Scheinker Disease with the P102L Mutation - Monaco, Salvatore; Fiorini, Michele; Farinazzo, Alessia; Ferrari, Sergio; Gelati, Matteo; Piccardo, Pedro; Zanusso, Gianluigi; Ghetti, Bernardino (PloS one, 2012)
Keywords: Adult; Aged; Alleles; Antibodies; Biology; Centrifugation, Density Gradient; Creutzfeldt-Jakob disease; Degeneration; Dementia; Diagnostic systems; Female; Fragmentation; Gerstmann-Straussler-Scheinker Disease - genetics; Gerstmann-Straussler-Scheinker Disease - metabolism; Humans; Immunohistochemistry; Immunohistochemistry - methods; Insomnia; Isoelectric Focusing; Isoforms; Laboratories; Leucine; Male; Medicine; Middle Aged; Models, Genetic; Morphology; Mutation; Neuropathology; Paraffin; Peptide Hydrolases - metabolism; Peptides; Phenotype; Phenotypes; Point Mutation; Positron-Emission Tomography - methods; Prion protein; Proline; Propagation; Protease; Protein Conformation; Protein purification; Proteinase; Proteins; Proteolysis; PrPSc Proteins - genetics; Separation techniques; Sucrose - chemistry; Transgenic animals; Western blotting; Italy; Indiana; Monaco; United States--US; Indianapolis Indiana