Pathogens 2013, 2(3), 457-471; doi:10.3390/pathogens2030457

Prions in Variably Protease-Sensitive Prionopathy: An Update

1,2,3,4,5,6,* email, 1,3email, 1email, 1email, 7email and 8email
Received: 12 June 2013; in revised form: 28 June 2013 / Accepted: 2 July 2013 / Published: 5 July 2013
(This article belongs to the Special Issue Prions)
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract: Human prion diseases, including sporadic, familial, and acquired forms such as Creutzfeldt-Jakob disease (CJD), are caused by prions in which an abnormal prion protein (PrPSc) derived from its normal cellular isoform (PrPC) is the only known component. The recently-identified variably protease-sensitive prionopathy (VPSPr) is characterized not only by an atypical clinical phenotype and neuropathology but also by the deposition in the brain of a peculiar PrPSc. Like other forms of human prion disease, the pathogenesis of VPSPr also currently remains unclear. However, the findings of the peculiar features of prions from VPSPr and of the possible association of VPSPr with a known genetic prion disease linked with a valine to isoleucine mutation at residue 180 of PrP reported recently, may be of great importance in enhancing our understanding of not only this atypical human prion disease in particular, but also other prion diseases in general. In this review, we highlight the physicochemical and biological properties of prions from VPSPr and discuss the pathogenesis of VPSPr including the origin and formation of the peculiar prions.
Keywords: prions; prion protein; prion disease; Creutzfeldt-Jakob disease (CJD); variably protease-sensitive prionopathy (VPSPr); Gerstmann-Sträussler-Scheinker (GSS); mutation; proteinase K; antibody; glycosylation; glycoform-selective prion formation; transmissibility
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MDPI and ACS Style

Zou, W.-Q.; Gambetti, P.; Xiao, X.; Yuan, J.; Langeveld, J.; Pirisinu, L. Prions in Variably Protease-Sensitive Prionopathy: An Update. Pathogens 2013, 2, 457-471.

AMA Style

Zou W-Q, Gambetti P, Xiao X, Yuan J, Langeveld J, Pirisinu L. Prions in Variably Protease-Sensitive Prionopathy: An Update. Pathogens. 2013; 2(3):457-471.

Chicago/Turabian Style

Zou, Wen-Quan; Gambetti, Pierluigi; Xiao, Xiangzhu; Yuan, Jue; Langeveld, Jan; Pirisinu, Laura. 2013. "Prions in Variably Protease-Sensitive Prionopathy: An Update." Pathogens 2, no. 3: 457-471.

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