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I.Benilova@prion.ucl.ac.uk Tel: 020 7679 5094 Courtauld Building, Room G01 • • Ìý

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Prion disease is a fatal transmissible neurodegenerative disease characterized by cerebral deposition of misfolded prion protein and neuronal loss. Studies in wild type and transgenic mouse models of prion disease suggest that prion propagation in brain involves two distinct phases. We hypothesize that once prion propagation saturates there is a mechanistic switch from autocatalytic production of infectivity to a toxic pathway where prions act as a catalytic surface for the production of toxic species. In Parmjit Jat’s group, we are developing multiparametric cell-based assays to investigate the precise temporal relationship between infectivity and toxicity in a mouse model of scrapie.

Benilova, I.,* Reilly, M.*, Terry, C., Wenborn, A., Schmidt, C., Marinho, A., Risse, E., Al-Doujaily, H., Wiggins De Oliveira, M., Sandberg, M., Wadsworth, J., Jat, P., Collinge, J. (2020) PNASÌý117Ìý(38), 23815-23822. * denotes equal contribution

Cerofolini, L., Ravera, E., Bologna, S., Wiglenda, T., Boddrich, A., Purfurst, B.,ÌýBenilova, I., Korsak, M., Gallo, G., Rizzo, D., Gonnelli, L., Fragai, M., De Strooper, B., Wanker, E., Luchinat, C. (2020) Chem CommÌý56, 8830-8833.

Philiastides, A., Ribes, J.M., Yip, D.C., Schmidt, C.,ÌýBenilova, I.,ÌýKlohn P.K. (2019) VirusesÌý11 (10), 888.

Benilova, I., Gallardo, R., Ungureanu, A. A., Castillo Cano, V., Snellinx, A., Ramakers, M., Bartic, C., Rousseau, F., Schymkowitz, J., and De Strooper, B. (2014) ÌýJBC 289, 30977–30989.

Benilova, I., Karran, E., and De Strooper, B. (2012) Nature Neuroscience 15, 349-357

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