NR AOTA
AU Giaccone,G.; Mangieri,M.; Capobianco,R.; Limido,L.; Di Fede,G.; Suardi,S.; Fociani,P.; Carsana,L.; Bugiani,O.; Tagliavini,F.
TI The involvement of the retina is a consistent finding in sporadic Creutzfeldt-Jakob disease
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Poster session - DG-60
PT Konferenz-Poster
AB Creutzfeldt-Jakob disease (CJD) is marked by the presence of the protease-resistant prion protein (PrPres) in the brain. We analyzed for PrPres the retina of 30 sporadic CJD patients (PRNP codon 129 polymorphism: MM n=22, VV n=5, MV n=3) and 15 patients with other neurological diseases. The diagnosis of sporadic CJD was definite on the basis of neuropathological findings, presence of cerebral PrPres deposition by immunohistochemistry and Western blot analysis, absence of PRNP mutations. The ocular tissue and optic nerves were collected at autopsy and the samples were fixed in Carnoy solution or frozen. Before immunohistochemistry with 3F4 antibody, the sections were pretreated with proteinase K and guanidine thiocyanate. PrPres immunoreactivity was detected in the retina of all 30 sporadic CJD patients, but not in control patients. In CJD, PrPres was localized in the outer and inner plexiform layers, with a coarser granular pattern in the former, without differences between patients with diverse polymorphism at codon 129 of PRNP. The pattern of PrP-immunoreactivity was similar to that obtained with antibodies to the presynaptic marker synaptophysin. Western blot analysis confirmed the presence of PrPres, whose relative amount, protease-resistance and molecular weight corresponded to those of the brain. No PrPres was detected in the optic nerves either by immunohistochemistry or immunoblot analysis. Our data indicate that PrPres deposition in the retina is a consistent event in sporadic CJD.
AD Giorgio Giaccone, Michela Mangieri, Raffaella Capobianco, Lucia Limido, Giuseppe Di Fede, Silvia Suardi, Orso Bugiani, Fabrizio Tagliavini, Istituto Nazionale Neurologico Carlo Besta, Italy; Paolo Fociani, Luca Carsana, Università di Milano (Ospedale Luigi Sacco), Italy
SP englisch
PO Deutschland