Prion strain characterization of a novel subtype of Creutzfeldt-Jakob disease

Roberta Galeno; Michele Angelo Di Bari; Romolo Nonno; Franco Cardone; Marco Sbriccoli; Silvia Graziano; Loredana Ingrosso; Michele Fiorini; Angelina Valanzano; Giulia Pasini; Anna Poleggi; Ramona Vinci; Anna Ladogana; Maria Puopolo; Salvatore Monaco; Umberto Agrimi; Gianluigi Zanusso; Maurizio Pocchiari

doi:10.1128/JVI.02390-16

Prion strain characterization of a novel subtype of Creutzfeldt-Jakob disease

Roberta Galeno, Michele Angelo Di Bari, Romolo Nonno, Franco Cardone, Marco Sbriccoli, Silvia Graziano, Loredana Ingrosso, Michele Fiorini, Angelina Valanzano, Giulia Pasini, Anna Poleggi, Ramona Vinci, Anna Ladogana, Maria Puopolo, Salvatore Monaco, Umberto Agrimi, Gianluigi Zanusso, Maurizio Pocchiari

Istituto Superiore di Sanita'

Research output: Contribution to journal › Article

Abstract

In 2007, we reported a patient with an atypical form of Creutzfeldt- Jakob disease (CJD) heterozygous for methionine-valine (MV) at codon 129 who showed a novel pathological prion protein (PrP^TSE) conformation with an atypical glycoform (AG) profile and intraneuronal PrP deposition. In the present study, we further characterize the conformational properties of this pathological prion protein (PrP^TSE MV^AG), showing that PrP^TSE MV^AG is composed of multiple conformers with biochemical properties distinct from those of PrP^TSE type 1 and type 2 of MV sporadic CJD (sCJD). Experimental transmission of CJD-MV^AG to bank voles and genetargeted transgenic mice carrying the human prion protein gene (TgHu mice) showed unique transmission rates, survival times, neuropathological changes, PrP^TSE deposition patterns, and PrP^TSE glycotypes that are distinct from those of sCJD-MV1 and sCJD-MV2. These biochemical and experimental data suggest the presence of a novel prion strain in CJD-MV^AG.

Original language	English
Article number	e02390-16
Journal	Journal of Virology
Volume	91
Issue number	11
DOIs	https://doi.org/10.1128/JVI.02390-16
Publication status	Published - Jun 1 2017

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Keywords

Creutzfeldt-Jakob disease
Humanized mice
Prion strain
Prions

ASJC Scopus subject areas

Immunology
Virology

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Galeno, R., Di Bari, M. A., Nonno, R., Cardone, F., Sbriccoli, M., Graziano, S., Ingrosso, L., Fiorini, M., Valanzano, A., Pasini, G., Poleggi, A., Vinci, R., Ladogana, A., Puopolo, M., Monaco, S., Agrimi, U., Zanusso, G., & Pocchiari, M. (2017). Prion strain characterization of a novel subtype of Creutzfeldt-Jakob disease. Journal of Virology, 91(11), [e02390-16]. https://doi.org/10.1128/JVI.02390-16

Prion strain characterization of a novel subtype of Creutzfeldt-Jakob disease. / Galeno, Roberta; Di Bari, Michele Angelo ; Nonno, Romolo ; Cardone, Franco ; Sbriccoli, Marco ; Graziano, Silvia ; Ingrosso, Loredana; Fiorini, Michele; Valanzano, Angelina; Pasini, Giulia; Poleggi, Anna; Vinci, Ramona; Ladogana, Anna ; Puopolo, Maria; Monaco, Salvatore; Agrimi, Umberto; Zanusso, Gianluigi; Pocchiari, Maurizio.

In: Journal of Virology, Vol. 91, No. 11, e02390-16, 01.06.2017.

Research output: Contribution to journal › Article

Galeno, R, Di Bari, MA , Nonno, R , Cardone, F , Sbriccoli, M , Graziano, S , Ingrosso, L, Fiorini, M, Valanzano, A, Pasini, G, Poleggi, A, Vinci, R, Ladogana, A , Puopolo, M, Monaco, S, Agrimi, U, Zanusso, G & Pocchiari, M 2017, 'Prion strain characterization of a novel subtype of Creutzfeldt-Jakob disease', Journal of Virology, vol. 91, no. 11, e02390-16. https://doi.org/10.1128/JVI.02390-16

Galeno, Roberta ; Di Bari, Michele Angelo ; Nonno, Romolo ; Cardone, Franco ; Sbriccoli, Marco ; Graziano, Silvia ; Ingrosso, Loredana ; Fiorini, Michele ; Valanzano, Angelina ; Pasini, Giulia ; Poleggi, Anna ; Vinci, Ramona ; Ladogana, Anna ; Puopolo, Maria ; Monaco, Salvatore ; Agrimi, Umberto ; Zanusso, Gianluigi ; Pocchiari, Maurizio. / Prion strain characterization of a novel subtype of Creutzfeldt-Jakob disease. In: Journal of Virology. 2017 ; Vol. 91, No. 11.

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abstract = "In 2007, we reported a patient with an atypical form of Creutzfeldt- Jakob disease (CJD) heterozygous for methionine-valine (MV) at codon 129 who showed a novel pathological prion protein (PrPTSE) conformation with an atypical glycoform (AG) profile and intraneuronal PrP deposition. In the present study, we further characterize the conformational properties of this pathological prion protein (PrPTSE MVAG), showing that PrPTSE MVAG is composed of multiple conformers with biochemical properties distinct from those of PrPTSE type 1 and type 2 of MV sporadic CJD (sCJD). Experimental transmission of CJD-MVAG to bank voles and genetargeted transgenic mice carrying the human prion protein gene (TgHu mice) showed unique transmission rates, survival times, neuropathological changes, PrPTSE deposition patterns, and PrPTSE glycotypes that are distinct from those of sCJD-MV1 and sCJD-MV2. These biochemical and experimental data suggest the presence of a novel prion strain in CJD-MVAG.",

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AU - Sbriccoli, Marco

AU - Graziano, Silvia

AU - Ingrosso, Loredana

AU - Fiorini, Michele

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AU - Vinci, Ramona

AU - Ladogana, Anna

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10.1128/JVI.02390-16