Gerstmann-Straussler-Scheinker disease

Creutzfeldt-Jakob disease (CJD) New variant CJD Gerstmann-Straussler-Scheinker disease Fatal familial insomnia Kuru Prion protein Extracellular deposits... 

Human prion disease models have also been developed in mice [154,155]. Crossing the species barrier into an experimentally accessible animal system, the prions responsible for Creutzfeldt Jakob disease, new variant CJD, Gerstmann-Straussler-Scheinker disease, and fatal familial insomnia produce a reproducible time-dependent neuronal degeneration leading to death. 

The prion diseases are a closely related group of neuro-degenerative conditions which affect both humans and animals. They have previously been described as the subacute spongiform encephalopathies, slow virus diseases and transmissible dementias, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and the human prion diseases, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru. Prion diseases are... 

Salmona, M., Morbin, M., Massignan, T., Colombo, L., Mazzoleni, G., Capobianco, R., Diomede, L., Thaler, F., Mollica, L., Musco, G., Kourie,J. J., Bugiani, O., et al. (2003). Structural properties of Gerstmann-Straussler-Scheinker disease amyloid protein. J. Biol Chem. 278, 48146-48153. 

II. The Amyloid Peptides in Gerstmann-Straussler-Scheinker Disease... 

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