Novel prion protein conformation and glycotype in Creutzfeldt-Jakob disease

Zanusso, Gianluigi; Polo, A.; Farinazzo, Alessia; Nonno, R.; Cardone, F.; Di Bari, M.; Ferrari, Sergio; Principe, S.; Gelati, Matteo; Fasoli, E.; Fiorini, Michele; Prelli, F.; Frangione, B.; Tridente, Giuseppe; Bentivoglio Fales, Marina; Giorgi, A.; Schininà, M. E.; Maras, B.; Agrimi, U.; Rizzuto, Nicolo'; Pocchiari, M.; Monaco, Salvatore

doi:10.1001/archneur.64.4.595

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 previously unrecognized PrPSc tertiary structure lacking diglycosylated species. Genetic analysis revealed a wild-type prion protein gene. The prion agent responsible for this atypical phenotype was successfully passaged to bank voles. Conclusion To our knowledge, our results define a new human prion disorder characterized by intracellular accumulation of a novel type of pathological prion protein.

Titolo:	Novel prion protein conformation and glycotype in Creutzfeldt-Jakob disease
Autori:	ZANUSSO, Gianluigi Polo A. FARINAZZO, Alessia Nonno R. Cardone F. Di Bari M. FERRARI, Sergio Principe S. GELATI, Matteo Fasoli E. FIORINI, Michele Prelli F. Frangione B. TRIDENTE, Giuseppe BENTIVOGLIO, Marina Giorgi A. Schininà M. E. Maras B. Agrimi U. RIZZUTO, Nicolo' Pocchiari M. MONACO, Salvatore mostra contributor esterni nascondi contributor esterni
Data di pubblicazione:	2007
Rivista:	ARCHIVES OF NEUROLOGY
Abstract:	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 previously unrecognized PrPSc tertiary structure lacking diglycosylated species. Genetic analysis revealed a wild-type prion protein gene. The prion agent responsible for this atypical phenotype was successfully passaged to bank voles. Conclusion To our knowledge, our results define a new human prion disorder characterized by intracellular accumulation of a novel type of pathological prion protein.
Handle:	http://hdl.handle.net/11562/310561
Appare nelle tipologie:	01.01 Articolo in Rivista

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