NR AQAT
AU Giaccone,G.; Canciani,B.; Piccardo,P.; Tagliavini,F.; Ghetti,B.; Bugiani,O.
TI Topography of AT8 immunoreactivity in Gerstmann-Sträussler-Scheinker-disease, indiana kindred
QU Journal of Neuropathology and Experimental Neurology 1997; 56(N5): 588 Nr. 69
PT Meeting Abstract
VT Gerstmann-Sträussler-Scheinker disease (GSS) is an inherited prion disease characterised by cerebral prion protein (PrP) amyloid. In the Indiana kindred of GSS, linked to a mutation at codon 198 of the PRNP gene resulting in Phe>Ser substitution, PrP-amyloid coexists with neurofibrillary changes morphologically and antigenically similar to those of Alzheimer disease. Aim of this study was to analyse the topography of phosphorylated tau in GSS-F198S using AT8, a monoclonal antibody that recognises tau phosphorylated at serine 202 and threonine 205. Immunohistochemistry was carried out on l0-µm-thick coronal sections of the cerebral hemispheres and cerebellum from seven patients with GSS-F198S. AT8 immunoreactivity (AT8-ir) was consistently strong in frontal, temporal and parietal cortex, whereas it was more variable and less intense in the occipital cortex. In cortical regions where the burden of AT8-ir was higher, a dense network of labeled profiles permeated homogeneously the cortical thickness, whereas in less affected areas, AT8-ir involved selectively the inner layers. ATS-ir was present in the caudate nucleus and putamen of all patients, with a severity comparable to that found in the most affected areas of the cerebral cortex; it was less intense in the thalamus and pallidum, while it was absent in the cerebellum, in spite of the presence of the greatest amount of PrP-amyloid. ATS-ir was localised to neuronal perikarya and neuropil threads, with clustering of AT8-ir profiles surrounding PrP-amyloid deposits. The intensity of AT8-ir in the frontal and temporal cortex of GSS-F198S patients was similar to that found in Alzheimer patients, although the intralaminar distribution was different. Further, at variance with Alzheimer disease, the involvement of caudate nucleus and putamen was constantly severe in GSS F198S. Our results indicate that phosphorylated tau accumulates in the brain of patients with GSS-F198S with a typical pattern of anatomical distribution.
IN Beim Gerstmann-Sträussler-Scheinker-Syndrom vom Typ Indiana führt die Mutation von Phenylalanin zu Serin im Codon 198 neben der Prionkrankheit ähnlich wie bei Alzheimer zu neurofibrillären Veränderungen und einer Akkumulation des phosphorylierten tau-Proteins mit einem spezifischen Verteilungsmuster.
AD G Giaccone1, B. Canciani1, P. Piccardo2*, F Tagliavini1*, B. Ghetti2*, O Bugiani1* 1 Istituto Nazionale Neurologico Carlo Besta, Milano, Italy, 2 Indiana University School of Medicine, Indianapolis, IN, USA
ZR 0
PO USA
SP englisch
OR Prion-Krankheiten G