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.
| Original language | English |
|---|---|
| Article number | e02390-16 |
| Journal | Journal of Virology |
| Volume | 91 |
| Issue number | 11 |
| DOIs | |
| 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
Cite this
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
}
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 -