Logo image
Human platelets as a substrate source for the in vitro amplification of the abnormal prion protein (PrPSc) associated with variant Creutzfeldt-Jakob disease
Journal article   Peer reviewed

Human platelets as a substrate source for the in vitro amplification of the abnormal prion protein (PrPSc) associated with variant Creutzfeldt-Jakob disease

M. Jones, A.H. Peden, H. Yull, D. Wight, M.T. Bishop, C.V. Prowse, M.L. Turner, J.W. Ironside, I.R. MacGregor and M.W. Head
Transfusion, Vol.49(2), pp.376-384
2009
url
Link to Published Version *Subscription may be requiredView

Abstract

BACKGROUND: Four recent cases of transfusion‐related transmission of variant Creutzfeldt‐Jakob disease (vCJD) highlight the need to develop a highly sensitive and specific screening test to detect infectivity in the blood of asymptomatic infected individuals. Protein misfolding cyclic amplification (PMCA), a method for the amplification of minute amounts of disease‐associated abnormal prion protein (PrPSc) to readily detectable levels, could be incorporated into such a test provided that a suitable substrate source for routine use in human PMCA reactions can be found. STUDY DESIGN AND METHODS: With the use of seed sources from individuals with variant and sporadic CJD, the use of human platelets (PLTs) as a PMCA substrate source was evaluated. The effects of seed/substrate prion protein gene (PRNP) codon 129 genotype compatibility on amplification efficiency and freeze‐thaw on a substrate's ability to support amplification and the degree of amplification achieved by serial PMCA (sPMCA) were investigated. RESULTS: Seed/substrate PRNP codon 129 compatibility was found to have a major influence on PrPSc amplification efficiency. Individual substrates, of the same PRNP codon 129 genotype, could be pooled and stored frozen for use in subsequent PMCA reactions. A consistent 10‐fold increase in PrPSc detection sensitivity was achieved after each round of sPMCA, resulting in a 10,000‐fold increase in detection sensitivity after four rounds, with no evidence of de novo PrPSc production detected in the unseeded PLT substrate. CONCLUSIONS: Providing issues of seed/substrate PRNP codon 129 compatibility are taken into consideration human PLTs are a suitable, readily available, renewable substrate source for use in human PMCA applications.

Details

UN Sustainable Development Goals (SDGs)

This output has contributed to the advancement of the following goals:

#3 Good Health and Well-Being

Source: InCites

Metrics

InCites Highlights

These are selected metrics from InCites Benchmarking & Analytics tool, related to this output

Citation topics
1 Clinical & Life Sciences
1.52 Neurodegenerative Diseases
1.52.992 Prion Pathogenesis
Web Of Science research areas
Hematology
ESI research areas
Clinical Medicine
Logo image