Prions in Variably Protease-Sensitive Prionopathy: An Update

Prions in Variably Protease-Sensitive Prionopathy: An Update

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-sensi...

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Bibliographic Details
Main Authors: Laura Pirisinu, Jan Langeveld, Jue Yuan, Xiangzhu Xiao, Wen-Quan Zou, Pierluigi Gambetti
Format: Article
Language:English
Published: MDPI AG 2013-07-01
Series:Pathogens
Subjects:
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
Online Access:http://www.mdpi.com/2076-0817/2/3/457

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