Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu

Tamaki Muramoto, Tomoyuki Tanaka, Noritoshi Kitamoto, Chiharu Sano, Yushi Hayashi, Tomoko Kutomi, Chikao Yutani, Tetsuyuki Kitamoto

Research output: Contribution to journalArticlepeer-review

23 Citations (Scopus)

Abstract

Two monoclonal antibodies that specifically detect human prion protein (PrP) were developed. The epitope of both antibodies was mapped using fusion proteins of glutathione-S-transferase and PrP peptides to the C-terminal region encompassing the polymorphic 219 residue. The antibodies recognized human PrP with 219Glu but not that with 219Lys. The unique property of the antibodies was utilized to determine the allelic origin of abnormal PrP deposited in the brain of a patient with Gerstmann-Straussler syndrome (GSS) with 102Leu/219Lys encoded by the same allele. Abnormal PrP was exclusively of mutant allelic origin, suggesting that 219Lys may be permissive to the formation of abnormal PrP in GSS. The antibodies may help to explore the relationship of 219Glu/Lys polymorphism to the pathogenesis of human prion diseases. Copyright (C) 2000 Elsevier Science Ireland Ltd.

Original languageEnglish
Pages (from-to)179-182
Number of pages4
JournalNeuroscience Letters
Volume288
Issue number3
DOIs
Publication statusPublished - 2000 Jul 21

Keywords

  • Epitope
  • Gerstmann-Straussler syndrome
  • Human
  • Monoclonal antibody
  • Polymorphism
  • Prion protein

ASJC Scopus subject areas

  • Neuroscience(all)

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