A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-Sträussler syndrome.

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.

journal_name

Neurology

journal_title

Neurology

authors

Kitamoto T,Yamaguchi K,Doh-ura K,Tateishi J

doi

10.1212/wnl.41.2_part_1.306

subject

Has Abstract

pub_date

1991-02-01 00:00:00

pages

306-10

issue

2 ( Pt 1)

eissn

0028-3878

issn

1526-632X

journal_volume

41

pub_type

杂志文章
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    pub_type: 评论,社论

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