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

T. Kitamoto, K. Yamaguchi, K. Doh-Ura, J. Tateishi

研究成果: Article査読

37 被引用数 (Scopus)

抄録

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.

本文言語English
ページ(範囲)306-310
ページ数5
ジャーナルNeurology
41
2
DOI
出版ステータスPublished - 1991 2
外部発表はい

ASJC Scopus subject areas

  • 臨床神経学

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