Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Sträussler-Scheinker syndrome

J. Tateishi, T. Kitamoto, K. Doh-Ura, Y. Sakaki, G. Steinmetz, C. Tranchant, J. M. Warter, N. Heldt

Research output: Contribution to journalArticlepeer-review

84 Citations (Scopus)

Abstract

Using immunostaining with anti-prion protein (PrP) antiserum, we detected numerous kuru plaques in the brain of a 24-year-old man with Gerstmann-Straussler-Scheinker syndrome. Immunoreactivity on Western blotting of the protease-resistant PrP fraction from the frozen brain was weak. PrP gene analysis showed substitution of alanine to valine in codon 117 but no substitution in codon 102. As the experimental transmission of the disease to mice was negative, a pathogen of a relatively low infectivity may cause the disease in predisposed family members.

Original languageEnglish
Pages (from-to)1578-1581
Number of pages4
JournalNeurology
Volume40
Issue number10
DOIs
Publication statusPublished - 1990 Oct
Externally publishedYes

ASJC Scopus subject areas

  • Clinical Neurology

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