NR ATGK
AU Giaccone,G.; Mangieri,M.; Hauw,J.J.; Haik,S.; Limido,L.; Capobianco,R.; Fociani,P.; Bugiani,O.; Tagliavini,F.
TI Tauopathy in variant Creutzfeldt-Jakob disease
QU International Conference - Prion 2005: Between fundamentals and society's needs - 19.10.-21.10.2005, Congress Center Düsseldorf - Oral sessions ORAL-41
PT Konferenz-Vortrag
AB Intraneuronal accumulation of hyperphosphorylated tau (phospho-tau) is not part of the neuropathological picture of Creutzfeldt-Jakob disease (CJD) while occurs in several genetic variants of Gerstmann-Sträussler-Scheinker disease (GSS). Aim of this study was to investigate whether phospho-tau-related changes are present in variant CJD (vCJD) that shares with GSS abundant PrP deposition in amyloid form. Immunohistochemistry was carried out on brain sections from five vCJD patients (age at death 26-57 years, disease duration 12-27 months) using antibodies 3F4 and 6H4 to prion protein (PrP), antibodies AT8 (epitope at residues 199-205) and AD2 (epitope at residues 396-404) to phosphorylation-dependent epitopes of tau, and antibodies to A-ß and A-ß precursor protein (A-ßPP). A large number of phospho-tau immunoreactive round or rod-shaped profiles, often clustered around PrP amyloid deposits or vacuoles of spongiosis, were observed in the cerebral cortex of all patients. Furthermore, rare phospho-tau immunolabeled neuronal perikaria and dendrites were present in the cerebral cortex and in the cerebellum. In addition, APP immunoreactive enlarged cell processes were abundant in association with PrP amyloid in the cerebral cortex, basal ganglia and granular layer of the cerebellum, while A-ß immunostaining was absent. Our results indicates that tau and APP pathology is a consistent feature of vCJD, likely representing the reaction of neurites to PrP amyloid.
AD G.Giaccone, M.Mangieri, L.Limido, R.Capobianco, P.Fociani, O.Bugiani, F.Tagliavini, Istituto Nazionale Neurologico Carlo Besta, Italy; J.-J.Hauw, S.Haïk, INSERM, Salpêtrière Hospital, France
SP englisch
PO Deutschland