Article
Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Sträussler-Scheinker syndrome.
Neurology - 1 Oct 1990
Tateishi J, Kitamoto T, Doh-ura K, Sakaki Y, Steinmetz G, Tranchant C, Warter J M, Heldt N
Abstract excerpt
Using immunostaining with anti-prion protein (PrP) antiserum, we detected numerous kuru plaques in the brain of a 24-year-old man with Gerstmann-Sträussler-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...
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