title: Standards for the assay of Creutzfeldt-Jakob disease specimens.
abstract: 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.
authors.affiliation: NIBSC, Blanche Lane, South Mimms, Potters Bar EN6 3QG, UK.
authors.forename: P
authors.initials: P
authors.lastname: Minor
authors.affiliation: National Transfusion Microbiology Reference Lab, National Blood Service, Colindale Avenue, London NW9 5BG, UK.
authors.forename: J
authors.initials: J
authors.lastname: Newham
authors.affiliation: NIBSC, Blanche Lane, South Mimms, Potters Bar EN6 3QG, UK.
authors.forename: N
authors.initials: N
authors.lastname: Jones
authors.affiliation: University of Toronto, CRND, Tanz Building, 6 Queen's Park Crescent, West Toronto, Ontario, Canada, M5S 3H2.
authors.forename: C
authors.initials: C
authors.lastname: Bergeron
authors.affiliation: VA Medical Centre, Mailstop 151, Room 3C-128, 10N Green Street, Baltimore, MD 21201, USA.
authors.forename: L
authors.initials: L
authors.lastname: Gregori
authors.affiliation: FDA CBER, Division of Emerging and Transfusion-transmitted Diseases, OBRR, CBER, FDA HFM310, 1401 Rockville Pike, Rockville, MD 20852-1448, USA.
authors.forename: D
authors.initials: D
authors.lastname: Asher
authors.affiliation: Sanquin Research, Plesmanlaan 125, PO BOX 9190, 1066 AD Amsterdam, The Netherlands.
authors.forename: F
authors.initials: F
authors.lastname: van Engelenburg
authors.affiliation: Institute of Neurology, Medical University of Vienna, Austrian Reference Centre for Human Prion Diseases, AKH 4J, A-1097 Vienna, Austria.
authors.forename: T
authors.initials: T
authors.lastname: Stroebel
authors.affiliation: Aventis Behring GmbH, Postfach 1230, 35002 Marburg, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Vey
authors.affiliation: Department of Clinical Veterinary Medicine, University of Cambridge, Madingley Road, Cambridge CB3 0EG, UK.
authors.forename: G
authors.initials: G
authors.lastname: Barnard
authors.affiliation: National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK.
authors.forename: M
authors.initials: M
authors.lastname: Head
authors.affiliation: NIBSC, Blanche Lane, South Mimms, Potters Bar EN6 3QG, UK.
authors.lastname: The Who Working Group On International Reference Materials For The Diagnosis And Study Of Transmissible Spongiform Encephalopathies
mesh_terms: D001923:Brain Chemistry; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis*; D006801:Humans; D015151:Immunoblotting; D018620:PrPSc Proteins / Q000032:analysis*
chemical_list: D018620:PrPSc Proteins