NR AGNS
AU Kitamoto,T.; Yamaguchi,K.; Doh-ura,K.; Tateishi,J.
TI A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-Sträussler syndrome
QU Neurology 1991 Feb; 41(2(1)): 306-10
PT journal article
AB 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.
MH Amino Acid Sequence; Chromatography, High Pressure Liquid/methods; Creutzfeldt-Jakob Syndrome/metabolism/pathology; Gerstmann-Sträussler-Scheinker Disease/*metabolism/pathology; Human; PrPsc Proteins; Prions/metabolism; Support, Non-U.S. Gov't; Variation (Genetics); Viral Proteins/genetics/*metabolism
AD Department of Neuropathology, Kyushu University, Fukuoka, Japan.
SP englisch
PO USA