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

Michael Jones, Alexander H Peden, Darren Wight, Christopher Prowse, Ian Macgregor, Jean Manson, Marc Turner, James W Ironside, Mark W Head

Research output: Contribution to journalArticlepeer-review


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.
Original languageEnglish
Pages (from-to)1783-6
Number of pages4
Issue number18
Publication statusPublished - Dec 2008


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