Journal article
A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-Sträussler syndrome
Neurology, Vol.41(2), pp.306-310
1991
DOI: 10.1212/WNL.41.2_Part_1.306
PMID: 1671530
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
Details
- Title: Subtitle
- A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-Sträussler syndrome
- Creators
- T KITAMOTO - Kyushu univ., neurological inst., dep. neuropathology, Higashi-ku, Fukuoka 812, JapanK YAMAGUCHI - Kyushu univ., neurological inst., dep. neuropathology, Higashi-ku, Fukuoka 812, JapanK DOH-URA - Kyushu univ., neurological inst., dep. neuropathology, Higashi-ku, Fukuoka 812, JapanJ TATEISHI - Kyushu univ., neurological inst., dep. neuropathology, Higashi-ku, Fukuoka 812, Japan
- Resource Type
- Journal article
- Publication Details
- Neurology, Vol.41(2), pp.306-310
- Publisher
- Lippincott Williams & Wilkins; Hagerstown, MD
- DOI
- 10.1212/WNL.41.2_Part_1.306
- PMID
- 1671530
- ISSN
- 0028-3878
- eISSN
- 1526-632X
- Language
- English
- Date published
- 1991
- Academic Unit
- Iowa Neuroscience Institute; Anesthesia; Neuroscience and Pharmacology
- Record Identifier
- 9984071998802771
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