Gerstmann-straussler-scheinker disease (Prnp p102l): Amyloid deposits are best recognized by antibodies directed to epitopes in prp region 90-165

Pedro Piccardo, Bernardino Ghetti, Dennis W. Dickson, Harry V. Vinters, Giorgio Giaccone, Orso Bugiani, Fabrizio Tagliavini, Katherine Young, Stephen R. Dlouhy, Charles Seiler, Carrie K. Jones, Alice Lazzarini, Lawrence I. Golbe, Thomas R. Zimmerman, Susan L. Perlman, Donald C. McLachlan, Peter H.St George-Hyslop, Anne Lennox

Research output: Contribution to journalArticle

36 Scopus citations

Abstract

Gerstmann-Straussler-Scheinker (GSS) disease is a familial neurological disorder pathologically characterized by accumulation of prion protein (PrP) in the form of fibrillary and non-fibrillary deposits within the cerebrum and cerebellum. We have studied two patients in whom the disease is caused by a leucine for proline amino acid substitution at residue 102 of PrP. In both patients, the neuropathologic findings are similar, consisting of spongiform changes, amyloid deposits, and gliosis. To investigate the antigenic profile of PrP deposits, we used antibodies raised against several peptides that correspond to segments of the N-terminus, repeat region, midregion, and C-terminus of PrP. By immunohistochemistry, PrP amyloid cores are best labeled by antibodies directed to epitopes spanning PrP residues 90-165. In GSS disease caused by a substitution of thymine to cytosine at PRNP codon 198 (Indiana kindred), the major amyloidogenic peptide spans residues 58-150; therefore, in these two genetic forms of GSS disease, amyloid may be composed of different peptides. 1995 by the American Association of Neuropathologists.

Original languageEnglish (US)
Pages (from-to)790-801
Number of pages12
JournalJournal of Neuropathology and Experimental Neurology
Volume54
Issue number6
DOIs
StatePublished - Nov 1995

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Keywords

  • Amyloid
  • Antibodies
  • Gerstmann-Straussler-Scheinker disease
  • Immunohistochemistry
  • PrP peptides
  • Prion protein (PrP)

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

Cite this

Piccardo, P., Ghetti, B., Dickson, D. W., Vinters, H. V., Giaccone, G., Bugiani, O., Tagliavini, F., Young, K., Dlouhy, S. R., Seiler, C., Jones, C. K., Lazzarini, A., Golbe, L. I., Zimmerman, T. R., Perlman, S. L., McLachlan, D. C., George-Hyslop, P. H. S., & Lennox, A. (1995). Gerstmann-straussler-scheinker disease (Prnp p102l): Amyloid deposits are best recognized by antibodies directed to epitopes in prp region 90-165. Journal of Neuropathology and Experimental Neurology, 54(6), 790-801. https://doi.org/10.1097/00005072-199511000-00006