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 › peer-review

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

Keywords

Creutzfeldt-Jakob disease
Humanized mice
Prion strain
Prions

ASJC Scopus subject areas

Immunology
Virology

Access to Document

10.1128/JVI.02390-16

Fingerprint Dive into the research topics of 'Prion strain characterization of a novel subtype of Creutzfeldt-Jakob disease'. Together they form a unique fingerprint.

Cite this

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 › peer-review

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.

@article{47e6087e1ad04151981ec7b71edbead7,

title = "Prion strain characterization of a novel subtype of Creutzfeldt-Jakob disease",

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.",

keywords = "Creutzfeldt-Jakob disease, Humanized mice, Prion strain, Prions",

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

year = "2017",

month = jun,

day = "1",

doi = "10.1128/JVI.02390-16",

language = "English",

volume = "91",

journal = "Journal of Virology",

issn = "0022-538X",

publisher = "American Society for Microbiology",

number = "11",

}

TY - JOUR

T1 - Prion strain characterization of a novel subtype of Creutzfeldt-Jakob disease

AU - Galeno, Roberta

AU - Di Bari, Michele Angelo

AU - Nonno, Romolo

AU - Cardone, Franco

AU - Sbriccoli, Marco

AU - Graziano, Silvia

AU - Ingrosso, Loredana

AU - Fiorini, Michele

AU - Valanzano, Angelina

AU - Pasini, Giulia

AU - Poleggi, Anna

AU - Vinci, Ramona

AU - Ladogana, Anna

AU - Puopolo, Maria

AU - Monaco, Salvatore

AU - Agrimi, Umberto

AU - Zanusso, Gianluigi

AU - Pocchiari, Maurizio

PY - 2017/6/1

Y1 - 2017/6/1

N2 - 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.

AB - 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.

KW - Creutzfeldt-Jakob disease

KW - Humanized mice

KW - Prion strain

KW - Prions

UR - http://www.scopus.com/inward/record.url?scp=85019245072&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85019245072&partnerID=8YFLogxK

U2 - 10.1128/JVI.02390-16

DO - 10.1128/JVI.02390-16

M3 - Article

C2 - 28298604

AN - SCOPUS:85019245072

VL - 91

JO - Journal of Virology

JF - Journal of Virology

SN - 0022-538X

IS - 11

M1 - e02390-16

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Cite this