Abstract:
BACKGROUND:A highly sensitive dissociation-enhanced lanthanide fluoroimmunoassay (DELFIA) and flow cytometry techniques have previously been developed and employed to characterize soluble cellular prion protein (PrP(c)) expression in whole blood and separated components from healthy adult blood donors. No previous studies with these techniques have evaluated the concentration and expression of PrP in the blood of patients with variant Creutzfeldt-Jakob disease (vCJD). STUDY DESIGN AND METHODS:For blood from vCJD patients, sporadic CJD (sCJD) patients, non-CJD neurological controls, and healthy adults, PrP(c) was measured by DELFIA and cell-associated PrP was measured by flow cytometry. RESULTS:DELFIA analysis identified a significant reduction in the concentration of PrP(c) in the whole blood of vCJD (p = 0.012) and non-CJD neurological patients (p = 0.0004) compared with healthy adults. A significant elevation was found in plasma PrP(c) in sCJD patients compared with healthy adult (p = 0.022) and neurological controls (p = 0.050). Flow cytometry found no significant differences between groups in expression of PrP on platelets and lymphocytes, nor in sensitivity of cellular PrP to proteinase K. Neurological controls show significantly less PrP on red cells than healthy adults. CONCLUSION:There are differences in free and cell-associated PrP found in blood of CJD patients and control groups, some of which might be useful with other tests in disease profiling as an aid to diagnoses.
journal_name
Transfusionjournal_title
Transfusionauthors
Fagge T,Barclay GR,Macgregor I,Head M,Ironside J,Turner Mdoi
10.1111/j.0041-1132.2005.04342.xsubject
Has Abstractpub_date
2005-04-01 00:00:00pages
504-13issue
4eissn
0041-1132issn
1537-2995pii
TRF04342journal_volume
45pub_type
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