An overview of the diagnostic tools.
Prions are unconventional infectious agents that cause fatal neurological illnesses such as Creutzfeldt-Jakob disease (CJD), bovine spongiform encephalopathy, and scrapie. Variant CJD can occur via blood transfusions. However, as no screening assay is available, uncertainties remain over the prevalence of vCJD in asymptomatic blood donors. Development of a diagnostic assay is therefore a primary objective. Little is known about the nature, distribution and level of infectivity in human blood and we have to rely on assumptions made from animal models. Ideally, two types of assays are required: a rapid high-throughput assay to routinely screen all blood donations and a confirmatory assay to ensure that all positive results from initial screening are true positives. Key event in prion disease is thought to be the conversion of normal cellular prion protein PrPc to a misfolded aggregated form termed PrP(TSE). This specific characteristic has been exploited to develop some tests.
Laboratoire R&D sécurité transfusionnelle et innovation diagnostique (TransDiag), établissement français du sang de Pyrénées-Méditerranée, 392, avenue du Professeur-Jean-Louis-Viala, 34184 Montpellier cedex 3, France. Electronic address: joliette.coste@efs.sante.fr.
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D000818:Animals; D000918:Antibody Specificity; D001782:Blood Donors; D058731:Blood Safety; D015153:Blotting, Western; D001923:Brain Chemistry; D007562:Creutzfeldt-Jakob Syndrome / Q000097:blood / Q000175:diagnosis; D004797:Enzyme-Linked Immunosorbent Assay; D000939:Epitopes / Q000276:immunology; D005188:False Negative Reactions; D005189:False Positive Reactions; D057166:High-Throughput Screening Assays / Q000379:methods; D006801:Humans; D008403:Mass Screening / Q000379:methods*; D008863:Microspheres; D010447:Peptide Hydrolases / Q000378:metabolism; D018621:PrPC Proteins / Q000737:chemistry / Q000276:immunology; D018620:PrPSc Proteins / Q000097:blood / Q000737:chemistry / Q000276:immunology; D017096:Prion Diseases / Q000097:blood / Q000175:diagnosis* / Q000662:veterinary; D011328:Prions / Q000097:blood* / Q000276:immunology; D011487:Protein Conformation; D017510:Protein Folding; D013010:Sonication; D013045:Species Specificity; D013997:Time Factors
D000939:Epitopes; D018621:PrPC Proteins; D018620:PrPSc Proteins; D011328:Prions; D010447:Peptide Hydrolases
Confirmation; Creutzfeldt-Jakob Disease; Detection; Détection; Maladie de Creutzfeldt-Jakob; PMCA; PrP(EST); PrP(TSE); QuIC,; vCJD; vMCJ