Scientific Publications by FDA Staff

J Gen Virol 2004 Jun;85(Pt 6):1777-1784

Standards for the assay of Creutzfeldt-Jakob disease specimens.

Minor P, Newham J, Jones N, Bergeron C, Gregori L, Asher D, Van Engelenburg F, Stroebel T, Vey M, Barnard G, Head M, The WHO Working Group on International Reference Materials for the Diagnosis and Study of Transmissible Spongiform Encephalopathies

Minor P, Natl Inst Biol Stand & Controls, Blanche Lane, Potters Bar EN6 3QG, Herts, England Natl Inst Biol Stand & Controls, Blanche Lane, Potters Bar EN6 3QG, Herts, England Natl Blood Serv, Natl Transfus Microbiol Reference Lab, London NW9 5BG, England Univ Toronto, CRND, W Toronto, ON M5S 3H2, Canada VA Med Ctr, Baltimore, MD 21201 USA US FDA, CBER, Div Emerging & Transfus Transmitted Dis, OBRR,HFM310, Rockville, MD 20852 USA Sanquin Res, NL-1066 AD Amsterdam, Netherlands Med Univ Vienna, Austrian Reference Ctr Human Pr Dis, Inst Neurol, A-1097 Vienna, Austria Aventis Behring GmbH, D-35002 Marburg, Germany Univ Cambridge, Dept Clin Vet Med, Cambridge CB3 0EG, England Western Gen Hosp, Natl Creutzfeldt Jakob Dis Surveillance Unit, Edinburgh EH4 2XU, Midlothian, Scotland

Assays for the agent of Creutzfeldt-Jakob disease (CJD) include measurement of infectivity in different animal systems, such as wild-type or transgenic mice, and detection of PrP(Sc) by different methods and formats. The various assays could be best calibrated against each other by use of uniform readily available materials, and samples of four human brains, two from sporadic CJD patients, one from a variant CJD patient and one from a non-CJD patient, have been prepared as 10 % homogenates dispensed in 2000 vials each for this purpose. Results of in vitro methods, particularly immunoblot assays, were compared in the first collaborative study described here. While dilution end-points varied, the minimum detectable volume was surprisingly uniform for most assays and differences in technical procedure, other than the sample volume tested, had no detectable systematic effect. The two specimens from sporadic CJD cases contained both type 1 and type 2 prion proteins in approximately equal proportions. The materials have been given the status of reference reagents by the World Health Organization and are available for further study and assessment of other in vitro or in vivo assay procedures.