Journal
NEUROREPORT
Volume 19, Issue 18, Pages 1783-1786Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/WNR.0b013e328318edfa
Keywords
model system; prion protein; protein misfolding cyclic amplification; sporadic Creutzfeldt-Jakob disease
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Funding
- Scottish National Blood Transfusion Service
- Chief Scientists Office of the Scottish Government [CBZ/4/357]
- European Network of Excellence NeuroPrion [FOOD-CT-2004-506579]
- Department of Health
- Scottish Government
- Medical Research Council [G0600953] Funding Source: researchfish
- MRC [G0600953] Funding Source: UKRI
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Prion protein type and codon 129 genotype are thought to be major determinants of susceptibility and phenotype in human prion diseases. Using an in-vitro system (protein misfolding cyclic amplification) we have attempted to model human prion protein conversion using the abnormal prion protein associated with each of the major sporadic Creutzfeldt-Jakob disease subtypes, in substrates containing the normal cellular form of the prion protein of each of the three possible human PRNP codon 129 polymorphic genotypes. The prion protein type is converted with fidelity in these amplification reactions, but the efficiency of conversion depends both on the methionine/valine polymorphic status of the sporadic Creutzfeldt-Jakob disease seed and substrate homogenate, and on the abnormal prion protein type. NeuroReport 19:1783-1786 (C) 2008 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.
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