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.
Subject(s)
Creutzfeldt-Jakob Syndrome/ethnology , Creutzfeldt-Jakob Syndrome/metabolism , Prions/metabolism , Adult , Aged , Asian People , Creutzfeldt-Jakob Syndrome/pathology , Germany/ethnology , Humans , Immunohistochemistry/methods , Japan/ethnology , Middle Aged , Periaqueductal Gray/metabolism , Synapses/metabolism , Synapses/ultrastructure , Tissue Distribution , White PeopleABSTRACT
We investigated paraffin-embedded brain sections from three patients with Gerstmann-Sträussler syndrome and three patients with Alzheimer's disease or senile dementia of Alzheimer type using anti-human prion protein antisera and anti-beta/A4 protein antisera after protein denaturation treatments. After incubation with guanidine-thiocyanate, trichloroacetate, and phenol, the immunoreactivity of kuru plaques and senile plaques was enhanced to the same level as the formic acid treatment. These treatments revealed small compact amyloid deposits, amyloid deposits surrounding the plaque cores, and diffuse plaques. Most of these chemicals changed the congophilia of both amyloids. It is possible that these treatments denature amyloid fibril proteins and break down the structure of amyloid fibrils, thus revealing buried epitopes.
