Gerstmann–Sträussler–Scheinker syndrome,fatal familial insomnia, and kuru: a review ofthese less common human transmissiblespongiform encephalopathies

@article{Collins2001GerstmannStrusslerScheinkerSF,
  title={Gerstmann–Str{\"a}ussler–Scheinker syndrome,fatal familial insomnia, and kuru: a review ofthese less common human transmissiblespongiform encephalopathies},
  author={S. Collins and C. McLean and C. Masters},
  journal={Journal of Clinical Neuroscience},
  year={2001},
  volume={8},
  pages={387-397}
}
  • S. Collins, C. McLean, C. Masters
  • Published 2001
  • Medicine
  • Journal of Clinical Neuroscience
  • Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal familial insomnia (FFI) and kuru constitute major human prion disease phenotypes. Each has been successfully transmitted in animal models and all are invariably fatal neurodegenerative disorders, with the brains of affected individuals harbouring variable amounts of an abnormal, protease-resistant form of the prion protein (PrPres), which is inextricably linked to pathogenesis and transmissibility. Classical… CONTINUE READING
    124 Citations
    Transmissible spongiform encephalopathies
    • 268
    iPS Cell Cultures from a Gerstmann-Sträussler-Scheinker Patient with the Y218N PRNP Mutation Recapitulate tau Pathology
    • 11
    • PDF
    Genetic PrP Prion Diseases.
    • 24
    • PDF
    Clinical diagnosis and differential diagnosis of CJD and vCJD
    • I. Zerr, S. Poser
    • Medicine
    • APMIS : acta pathologica, microbiologica, et immunologica Scandinavica
    • 2002
    • 119
    COVER 4_07

    References

    SHOWING 1-10 OF 100 REFERENCES
    Gerstmann‐Sträussler‐Scheinker Disease and the Indiana Kindred
    • 139
    Phenotypic Variability of Gerstmann-Straussler-Scheinker Disease is Associated with Prion Protein Heterogeneity
    • 193
    • PDF