2002-12-31-世界卫生组织-Proposal_to_establish_WHO_Reference_reagents_for_In_vitro_assays_of_CJD_specimens_18页_104kb

WHO Proposal for Establishing Reference Reagents for CJD Assays

Summary

The World Health Organization's Working Group aimed to create international reference reagents for in vitro assays of Creutzfeldt-Jakob Disease (CJD) specimens. Reference materials were prepared from homogenates of brain tissue: one normal human brain and cases from two sporadic CJD (sCJD) and one variant CJD (vCJD). All samples were homozygous for methionine at codon 129 of the PrP gene.

Six laboratories assessed the relative concentration of PrPres (abnormal prion protein) using Western blot assays. Relative PrPres concentrations showed one sCJD sample similar to vCJD, while the other sCJD had lower concentrations. Minimum detectable volumes of homogenate were consistent across labs, varying by dilution factors. The PrPres glycoform patterns confirmed subtypes in sCJD, with both samples containing mixed glycoforms. Materials are stored at –86°C and proposed as provisional WHO Reference Reagents for assay calibration.

Despite methodological variations, results supported the use of these materials for inter-laboratory comparisons. Future work includes expanding reference materials to include other CJD cases, PrP genotypes, lymphoid tissues, and rodent brain samples for broader TSE research.

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