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
Neurologyjournal_title
Neurologyauthors
Kitamoto T,Yamaguchi K,Doh-ura K,Tateishi Jdoi
10.1212/wnl.41.2_part_1.306subject
Has Abstractpub_date
1991-02-01 00:00:00pages
306-10issue
2 ( Pt 1)eissn
0028-3878issn
1526-632Xjournal_volume
41pub_type
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