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Distribution of prion protein in German patients with Creutzfeldt-Jakob disease is different from that in Japanese patients
Authors:R Doi-Yi  T Kitamoto  K Ogomori  P Mehraein  J Tateishi
Institution:(1) Department of Neuropsychiatry, Kumamoto University Medical School, Honjo 1-1-1, 860 Kumamoto, Japan;(2) Department of Neuropathology, Neurological Institute, Faculty of Medicine, Kyushu University, Japan;(3) Department of Neuropsychiatry, School of Medicine, Kyushu University, Japan;(4) Institute of Neuropathology, University of Munich, Germany
Abstract:We investigated the distribution of prion protein (PrP) in 14 German patients with sporadic Creutzfeldt-Jakob disease (CJD) and compared it with that observed in Japanese patients. Immunohistochemical study revealed diffuse gray matter stainings including synaptic structures in all cases. In addition, 4 patients showed plaque-type deposition which was very rarely observed among sporadic Japanese patients without known mutation of the PrP gene but with valine at codon 129. A higher incidence of PrP plaques in German sporadic CJD may be related to the racial difference in the PrP gene.
Keywords:Creutzfeld-Jakob disease (CJD)  Gerstmann-Strä  ussler-Scheinker syndrome (GSS)  Prion protein (PrP)  Genotypes  Phenotypes
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