Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assay

M. Jones; A.H. Peden; D. Wight; C. Prowse; I. MacGregor; J. Manson; M. Turner; J.W. Ironside; M.W. Head

doi:10.1097/WNR.0b013e328318edfa

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Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assay

Journal article

Peer reviewed

Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assay

M. Jones, A.H. Peden, D. Wight, C. Prowse, I. MacGregor, J. Manson, M. Turner, J.W. Ironside and M.W. Head

Neuroreport, Vol.19(18), pp.1783-1786

2008

DOI: https://doi.org/10.1097/WNR.0b013e328318edfa

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Abstract

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.

Details

Title: Effects of human PrPSc type and PRNP genotype in an in-vitro conversion assay
Authors/Creators: M. Jones (Author/Creator) - Roslin Institute
A.H. Peden (Author/Creator) - West Midlands Deanery
D. Wight (Author/Creator)
C. Prowse (Author/Creator)
I. MacGregor (Author/Creator)
J. Manson (Author/Creator) - Royal (Dick) School of Veterinary Studies
M. Turner (Author/Creator) - West Midlands Deanery
J.W. Ironside (Author/Creator) - Deanery of Molecular, Genetic and Population Health Sciences, Centre for Clinical Brain Sciences
M.W. Head (Author/Creator) - Deanery of Molecular, Genetic and Population Health Sciences, Centre for Clinical Brain Sciences
Publication Details: Neuroreport, Vol.19(18), pp.1783-1786
Publisher: Lippincott Williams & Wilkins
Identifiers: 991005542600807891
Murdoch Affiliation: Murdoch University
Language: English
Resource Type: Journal article

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Collaboration types: Domestic collaboration
Citation topics: 1 Clinical & Life Sciences; 1.52 Neurodegenerative Diseases; 1.52.992 Prion Pathogenesis
Web Of Science research areas: Neurosciences
ESI research areas: Neuroscience & Behavior