Seminarium Zakładu Biofizyki

Prion diseases are fatal neurodegenerative disorders caused by accumulation of the misfolded cellular prion conformer (PrPC) denoted as infection scrapie isoform (PrPSc). In inherited human prion disease, a mutation in the PrP gene (PRNP) possibly to favour spontaneous generation of PrPSc isoform in specific brain regions results in neuronal degeneration and death. The recently solved high-resolution 3D structures of two pathological PrPC mutants, Q212P and V210I, which represent truncated region of 90-231 human PRNP gene, will be presented. Close inspection of the experimental data and their comparison with the previously solved prion structures in solution from Prof. K. Wuthrich’s laboratory gives rise to possible mechanisms of PrPC → PrPSc conversion that leads to Gerstmann-Straussler-Scheinker (GSS) syndrome and Creutzfeldt-Jakob disease (CJD) in human.