Summary
Within the spectrum of sporadic human transmissible spongiform encephalopathies (TSEs), there is considerable diversity of disease phenotypes. At least part of this variation is thought to be on the basis of different “strains” of prions (the infectious agent). Tissue deposition of PrPres (the abnormal disease-associated conformation of the prion protein) is considered a hallmark of TSE pathology, and it can be visualized by Western blotting typically as three bands depicting the diglycosylated, monoglycosylated, and unglycosylated species. It is the mobility of the unglycosylated PrPres, and the relative abundance of the two glycosylated bands, along with the prion protein gene (PRNP) codon 129 genotype, that seem to correlate with distinct clinico-pathological profiles of sporadic Creutzfeldt-Jakob disease.
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References
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Acknowledgments
The ANCJDR is funded by the Commonwealth Department of Health and Ageing. We thank the families and clinicians for ongoing support in the surveillance of CJD in Australia. Original T1–T4 standards were supplied to the ANCJDR by the National Institute for Biological Standards and Control (NIBSC) as part of the World Health Organization Collaborative Nomenclature Study (2001–2002). Since then, Australian cases of Tl–3 glycotypes have been determined, and they are used as control samples for future glycotyping gels. We thank Dr. Victoria Lawson for the kind gift of the monoclonal mouse antibody 3F4.
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© 2008 Humana Press, a part of Springer Science + Business Media, LLC
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Lewis, V., Klug, G.M., Hill, A.F., Collins, S.J. (2008). Molecular Typing of PrPres in Human Sporadic CJD Brain Tissue. In: Hill, A.F. (eds) Prion Protein Protocols. Methods in Molecular Biology™, vol 459. Humana Press. https://doi.org/10.1007/978-1-59745-234-2_16
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DOI: https://doi.org/10.1007/978-1-59745-234-2_16
Publisher Name: Humana Press
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