Gerstmann-Straussler-Scheinker

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... 

EGTA ethyleneglycotetraacetic acid GSS Gerstmann-Straussler-Scheinker syndrome... 

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. 

Keywords Creutzfeldt Jakob disease Fatal insomnia Gerstmann-Straussler-Scheinker Kuru Neuroinvasion Prion Protein-only hypothesis. 

The human prion diseases include CJD, kuru, Gerstmann-Straussler-Scheinker (GSS), and fatal insomnia, which can be classified into three groups based on etiology (Table 29.1). 

Gerstmann-Straussler-Scheinker Acquired (-l ) 72 families Germ line mutations/ insertions in PRNP... 

Gerstmann-Straussler-Scheinker (GSS) syndrome is a prion disease finked to germ fine mutations or insertions in the human prion protein gene resulting in a neurodegenerative brain disorder. 

Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia, and kuru are fatal diseases of the central nervous system, known as transmissible subacute spongiform encephalopathies (193). Creutzfeldt-Jakob disease is the most common of these and has an incidence of 1 1000 000 in most countries. About 10% of those involved have a familial disposition, but most cases occur sporadically. There are similar diseases in sheep (scrapie), cattle (bovine spongiform encephalopathy), and other animal species. The cause of the... 

Kaneko K, Ball HL, Wille H, Zhang H, Groth D, Torchia M, Tremblay P, Safar J, Prusiner SB, DeArmond SJ, Baldwin MA, Cohen FE (2000) A synthetic peptide initiates Gerstmann-Straussler-Scheinker (GSS) disease in transgenic mice. J Mol Biol 295 997... 

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

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