A prion protein missense variant is integrated in kuru plaque cores in patients with gerstmann-sträussler syndrome

Tetsuyuki Kitamoto, K. Yamaguchi, Katsumi Doura, J. Tateishi

Research output: Contribution to journalArticlepeer-review

37 Citations (Scopus)

Abstract

Kuru plaques are the pathologic hallmark in Gerstmann-Sträussler syndrome (GSS). To demonstrate that prion protein (PrP) is a component of kuru plaque cores, we fractionated and sequenced kuru plaque core derived peptides, following digestion with Achromobacter lyticus protease I. We identified 3 PrP-derived peptides by reverse-phase high- performance liquid chromatography and found a fragment of digests derived from a missense variant of PrP. The variant PrP was also present in the prion rod fraction in patients with GSS. This substitution may play a major role in cerebral amyloidogenesis.

Original languageEnglish
Pages (from-to)306-310
Number of pages5
JournalNeurology
Volume41
Issue number2
Publication statusPublished - 1991 Jan 1
Externally publishedYes

ASJC Scopus subject areas

  • Clinical Neurology

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