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MeSH Review

Gerstmann-Straussler-Scheinker Disease

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Disease relevance of Gerstmann-Straussler-Scheinker Disease


Psychiatry related information on Gerstmann-Straussler-Scheinker Disease


High impact information on Gerstmann-Straussler-Scheinker Disease


Chemical compound and disease context of Gerstmann-Straussler-Scheinker Disease


Biological context of Gerstmann-Straussler-Scheinker Disease


Anatomical context of Gerstmann-Straussler-Scheinker Disease


Gene context of Gerstmann-Straussler-Scheinker Disease


  1. Prion protein amyloidosis. Ghetti, B., Piccardo, P., Frangione, B., Bugiani, O., Giaccone, G., Young, K., Prelli, F., Farlow, M.R., Dlouhy, S.R., Tagliavini, F. Brain Pathol. (1996) [Pubmed]
  2. Human prion diseases. Prusiner, S.B., Hsiao, K.K. Ann. Neurol. (1994) [Pubmed]
  3. A 7-kDa prion protein (PrP) fragment, an integral component of the PrP region required for infectivity, is the major amyloid protein in Gerstmann-Sträussler-Scheinker disease A117V. Tagliavini, F., Lievens, P.M., Tranchant, C., Warter, J.M., Mohr, M., Giaccone, G., Perini, F., Rossi, G., Salmona, M., Piccardo, P., Ghetti, B., Beavis, R.C., Bugiani, O., Frangione, B., Prelli, F. J. Biol. Chem. (2001) [Pubmed]
  4. Prion protein aggregation reverted by low temperature in transfected cells carrying a prion protein gene mutation. Singh, N., Zanusso, G., Chen, S.G., Fujioka, H., Richardson, S., Gambetti, P., Petersen, R.B. J. Biol. Chem. (1997) [Pubmed]
  5. Molecular biology and transgenetics of prion diseases. Prusiner, S.B. Crit. Rev. Biochem. Mol. Biol. (1991) [Pubmed]
  6. Familial dementia with PrP-positive amyloid plaques: a variant of Gerstmann-Sträussler syndrome. Nochlin, D., Sumi, S.M., Bird, T.D., Snow, A.D., Leventhal, C.M., Beyreuther, K., Masters, C.L. Neurology (1989) [Pubmed]
  7. Pathology of the vessels in cerebral amyloid angiopathy. Liberski, P.P., Barcikowska, M. Folia neuropathologica / Association of Polish Neuropathologists and Medical Research Centre, Polish Academy of Sciences. (1995) [Pubmed]
  8. Amyloid fibrils in Gerstmann-Sträussler-Scheinker disease (Indiana and Swedish kindreds) express only PrP peptides encoded by the mutant allele. Tagliavini, F., Prelli, F., Porro, M., Rossi, G., Giaccone, G., Farlow, M.R., Dlouhy, S.R., Ghetti, B., Bugiani, O., Frangione, B. Cell (1994) [Pubmed]
  9. A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy. Manson, J.C., Jamieson, E., Baybutt, H., Tuzi, N.L., Barron, R., McConnell, I., Somerville, R., Ironside, J., Will, R., Sy, M.S., Melton, D.W., Hope, J., Bostock, C. EMBO J. (1999) [Pubmed]
  10. Amyloid protein of Gerstmann-Sträussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58. Tagliavini, F., Prelli, F., Ghiso, J., Bugiani, O., Serban, D., Prusiner, S.B., Farlow, M.R., Ghetti, B., Frangione, B. EMBO J. (1991) [Pubmed]
  11. Creutzfeldt-Jakob disease patients with congophilic kuru plaques have the missense variant prion protein common to Gerstmann-Sträussler syndrome. Doh-ura, K., Tateishi, J., Kitamoto, T., Sasaki, H., Sakaki, Y. Ann. Neurol. (1990) [Pubmed]
  12. Cerebrospinal fluid levels of amyloid beta-protein precursor are low in Gerstmann-Sträussler-Scheinker disease, Indiana kindred. Farlow, M., Ghetti, B., Dlouhy, S., Giaccone, G., Bugiani, O., Tagliavini, F., Wagner, S. Neurology (1994) [Pubmed]
  13. Linkage of a prion protein missense variant to Gerstmann-Sträussler syndrome. Hsiao, K., Baker, H.F., Crow, T.J., Poulter, M., Owen, F., Terwilliger, J.D., Westaway, D., Ott, J., Prusiner, S.B. Nature (1989) [Pubmed]
  14. Gerstmann-Sträussler-Scheinker disease with mutation at codon 102 and methionine at codon 129 of PRNP in previously unreported patients. Young, K., Jones, C.K., Piccardo, P., Lazzarini, A., Golbe, L.I., Zimmerman, T.R., Dickson, D.W., McLachlan, D.C., St George-Hyslop, P., Lennox, A. Neurology (1995) [Pubmed]
  15. Familial prion disease mutation alters the secondary structure of recombinant mouse prion protein: implications for the mechanism of prion formation. Cappai, R., Stewart, L., Jobling, M.F., Thyer, J.M., White, A.R., Beyreuther, K., Collins, S.J., Masters, C.L., Barrow, C.J. Biochemistry (1999) [Pubmed]
  16. A Japanese family with a variant of Gerstmann-Sträussler-Scheinker disease. Tanaka, Y., Minematsu, K., Moriyasu, H., Yamaguchi, T., Yutani, C., Kitamoto, T., Furukawa, H. J. Neurol. Neurosurg. Psychiatr. (1997) [Pubmed]
  17. Immunolocalization of heparan sulfate proteoglycans to the prion protein amyloid plaques of Gerstmann-Straussler syndrome, Creutzfeldt-Jakob disease and scrapie. Snow, A.D., Wight, T.N., Nochlin, D., Koike, Y., Kimata, K., DeArmond, S.J., Prusiner, S.B. Lab. Invest. (1990) [Pubmed]
  18. Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Sträussler-Scheinker disease. Parchi, P., Chen, S.G., Brown, P., Zou, W., Capellari, S., Budka, H., Hainfellner, J., Reyes, P.F., Golden, G.T., Hauw, J.J., Gajdusek, D.C., Gambetti, P. Proc. Natl. Acad. Sci. U.S.A. (1998) [Pubmed]
  19. New variant prion protein in a Japanese family with Gerstmann-Sträussler syndrome. Furukawa, H., Kitamoto, T., Tanaka, Y., Tateishi, J. Brain Res. Mol. Brain Res. (1995) [Pubmed]
  20. Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Sträussler syndrome. Kitamoto, T., Ohta, M., Doh-ura, K., Hitoshi, S., Terao, Y., Tateishi, J. Biochem. Biophys. Res. Commun. (1993) [Pubmed]
  21. Gerstmann-Sträussler-Scheinker disease. II. Neurofibrillary tangles and plaques with PrP-amyloid coexist in an affected family. Ghetti, B., Tagliavini, F., Masters, C.L., Beyreuther, K., Giaccone, G., Verga, L., Farlow, M.R., Conneally, P.M., Dlouhy, S.R., Azzarelli, B. Neurology (1989) [Pubmed]
  22. Distinctive, rapid, and easy labeling of diffuse plaques in the Alzheimer brains by a new methenamine silver stain. Yamaguchi, H., Haga, C., Hirai, S., Nakazato, Y., Kosaka, K. Acta Neuropathol. (1990) [Pubmed]
  23. Neurofibrillary tangles of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease share antigenic determinants with those of Alzheimer disease. Giaccone, G., Tagliavini, F., Verga, L., Frangione, B., Farlow, M.R., Bugiani, O., Ghetti, B. Brain Res. (1990) [Pubmed]
  24. Reduced cortical choline acetyltransferase activity in Gerstmann-Sträussler syndrome. Robitaille, Y., Wood, P.L., Etienne, P., Lal, S., Finlayson, M.H., Gauthier, S., Nair, N.P. Prog. Neuropsychopharmacol. Biol. Psychiatry (1982) [Pubmed]
  25. The human prion diseases. A review with special emphasis on new variant CJD and comments on surveillance. Keohane, C. Clinical and experimental pathology. (1999) [Pubmed]
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