Inter-laboratory assessment of PrP Sc typing in creutzfeldt-jakob disease: A western blot study within the neuroprion consortium

Piero Parchi, Silvio Notari, Petra Weber, Heinz Schimmel, Herbert Budka, Isidre Ferrer, Stéphane Haik, Jean Jacques Hauw, Mark W. Head, James W. Ironside, Lucia Limido, Agustin Rodriguez, Thomas Ströbel, Fabrizio Tagliavini, Hans A. Kretzschmar

Research output: Contribution to journalArticle

Abstract

Molecular typing is of considerable importance for the surveillance and epidemiology of human transmissible spongiform encephalopathies (TSEs). It relies on the detection of distinct protease-resistant prion protein (PrP Sc) core fragments that differ in molecular mass and/or glycoform ratio. In this collaborative study, we tested the inter-laboratory agreement in TSE molecular typing. Sixteen characterized brain specimens from sporadic TSEs and variant Creutzfeldt-Jakob disease (vCJD) cases were distributed blindly to seven laboratories for molecular characterization by a defined protocol and classification. Agreement between laboratories in the classification of samples was excellent. In particular, there were no differences in the distinction between PrP Sc type 1, type 2A, and type 2B with one exception, which eventually was identified as a case with types 1 and 2 co-occurrence. This shows that the general technique and particular classification system used here are robust and represent a reliable basis for diagnostic and epidemiologic purposes. The subtle further distinction of subtypes among type 1 and type 2 groups requires high-sensitivity gel electrophoresis protocols that are unsuitable for routine diagnostic needs and must be reserved for research investigations. Further research is necessary on the identification and significance of co-occurrence of PrP Sc types 1 and 2 within one brain.

Original languageEnglish
Pages (from-to)384-391
Number of pages8
JournalBrain Pathology
Volume19
Issue number3
DOIs
Publication statusPublished - Jul 2009

Keywords

  • Classification
  • Dementia
  • Diagnosis
  • Prion
  • Strain typing

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine
  • Clinical Neurology

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    Parchi, P., Notari, S., Weber, P., Schimmel, H., Budka, H., Ferrer, I., Haik, S., Hauw, J. J., Head, M. W., Ironside, J. W., Limido, L., Rodriguez, A., Ströbel, T., Tagliavini, F., & Kretzschmar, H. A. (2009). Inter-laboratory assessment of PrP Sc typing in creutzfeldt-jakob disease: A western blot study within the neuroprion consortium. Brain Pathology, 19(3), 384-391. https://doi.org/10.1111/j.1750-3639.2008.00187.x