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Cambridge Immunology Network

 

Research

Prion diseases such as scrapie of sheep and goats, BSE of cattle and CJD of humans are transmissible neurodegenerative diseases, characterized by accumulation in the brain of subfibrillar and fibrillar forms of PrPSc, a disease-associated isomer of the host protein PrPC. In some cases, PrPSc may accumulate in peripheral lymphoid tissue prior to prion neuroinvasion of the CNS.

The research of our prion group is concerned with providing knowledge to try and answer some of the important questions relating to aspects of prion disease pathogenesis, diagnosis and therapeutic strategy. These questions include defining the nature of the infectious agent, determining the molecular basis of prion strain diversity, understanding the mechanism of protein misfolding as PrPC converts into PrPSc, and identification of molecular targets for diagnostic and potential therapeutic interventions. These aspects of PrP biology are being pursued in our attempts to identify and quantify abnormal PrP in the blood of individuals with pre-clinical prion disease. These studies underpin our efforts to develop an effective ante-mortem blood test for prion disease.

Our research on PrP involves collaborations with international and national experts in biochemistry, immunology, nanotechnology, protein chemistry and computational analysis of protein folding.

Publications

Key publications: 

Thackray AM, Zhang C, Arndt T, Bujdoso R. Cytosolic PrP can participate in prion-mediated toxicity. J Virol. 2014 May 7. [Epub ahead of print]

Beck KE, Thorne L, Lockey R, Vickery CM, Terry LA, Bujdoso R, Spiropoulos J. Strain typing of classical scrapie by transgenic mouse bioassay using protein misfolding cyclic amplification to replace primary passage. PLoS One. 2013;8(3):e57851. doi: 10.1371/journal.pone.0057851. 

Thackray, A.M., Lockey, R., Beck, K.E., Spiropoulos, J. & Bujdoso, R.  Evidence for co-infection of ovine prion strains in classical scrapie isolates. Journal of Comparative Pathology.  2012 Aug-Oct;147(2-3):316-29.

Thackray, A.M., Muhammad, F., Zhang, C., Di, Y., Jahn, T.R., Landgraf, M., Crowther, D.C., Evers, J.F, & Bujdoso, R.  Ovine PrP transgenic Drosophila show reduced locomotor activity and decreased survival. Biochemical Journal. 2012 444: 487-495.

Thackray, A.M., Muhammad, F., Zhang, C., Denyer, M., Spiropoulos, J., Crowther, D.C, & Bujdoso, R. Prion-induced toxicity in PrP transgenic Drosophila. Experimental and Molecular Pathology 2012. 92: 194-201

Thackray, A.M., Hopkins, L., Lockey, R., Spiropoulos, J. & Bujdoso, R.  Propagation of ovine prions from poor transmitter scrapie isolates in ovine PrP transgenic mice. Experimental and Molecular Pathology 2012. 92: 167-174.

Thackray, A.M., Hopkins, L., Lockey, R., Spiropoulos, J. & Bujdoso, R.  Emergence of multiple prion strains from single isolates of ovine scrapie. Journal of General Virology 2011 92: 1482-1491.

Dr Raymond   Bujdoso
Takes PhD students
Not available for consultancy

Affiliations

Classifications: 
Departments and institutes: 
Person keywords: 
diagnostic tests
prions
protein misfolding
heat shock proteins (hsp)
PrP
protein structure/folding
dendritic cells
interleukin
cytokines