Distinctive properties of plaque-type dura mater graft-associated Creutzfeldt-Jakob disease in cell-protein misfolding cyclic amplification

Laboratory Investigation; a Journal of Technical Methods and Pathology
Atsuko TakeuchiTetsuyuki Kitamoto

Abstract

There are two distinct subtypes of dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) with methionine homozygosity at codon 129 of the PRNP gene. The majority of cases is represented by a non-plaque-type (np-dCJD) resembling sporadic CJD (sCJD)-MM1 or -MV1, while the minority by a plaque-type (p-dCJD). p-dCJD shows distinctive phenotypic features, namely numerous kuru plaques and an abnormal isoform of prion protein (PrP(Sc)) intermediate in size between types 1 and 2. Transmission studies have shown that the unusual phenotypic features of p-dCJD are linked to the V2 prion strain that is associated with sCJD subtypes VV2 or -MV2. In this study, we applied protein misfolding cyclic amplification (PMCA) using recombinant human prion protein as a substrate and demonstrated that p-dCJD prions show amplification features that are distinct from those of np-dCJD. Although no amplification of np-dCJD prions was observed with either 129 M or 129 V substrate, p-dCJD prions were drastically amplified with the 129 V substrates, despite the PRNP codon 129 incompatibility between seed and substrate. Moreover, by using a type 2 PrP(Sc)-specific antibody not recognizing PrP(Sc) in p-dCJD, we found that type 2 products are generated d...Continue Reading

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Citations

Jul 3, 2017·Journal of Neurology, Neurosurgery, and Psychiatry·Simone BaiardiPiero Parchi
Jul 21, 2020·Emerging Infectious Diseases·Peter HermannInga Zerr
Jan 10, 2018·Acta Neuropathologica Communications·Ignazio CaliPierluigi Gambetti
Sep 22, 2020·Brain Communications·Atsuko TakeuchiTetsuyuki Kitamoto
Dec 28, 2018·Brain Pathology·Simone BaiardiPiero Parchi

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