NR AWJP
AU Mangieri,M.; Giaccone,G.; Limido,L.; Di Fede,G.; Suardi,S.; Capobianco,R.; Fociani,P.; Bugiani,O.; Tagliavini,F.
TI Sporadic Creutzfeldt-Jakob disease: PrPres is constantly present in the retina, and rarely in the optic nerve
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions PA-34
PT Konferenz-Poster
AB Creutzfeldt-Jakob disease (CJD) is marked by the presence of the protease-resistant prion protein (PrPres) in the brain. Studies of the retina and optic nerve in patients with CJD are scanty and on very small series of patients. We analysed ocular tissues of sporadic CJD patients (retina of 58 and optic nerve of 51), representing all combinations of PRNP codon 129 polymorphisms and PrPres types by Parchi, except VV1. Ocular tissue from 24 patients with other neurological diseases were used as controls. The ocular tissue was 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. In all cases of sCJD the retina showed immunoreactivity for PrPres localized in the inner and outer plexiform layers, with a synaptic type of labelling. No difference in the pattern of labeling was detected between CJD patients with different PRNP codon 129 polymorphisms and PrPres types in the brain. In all cases with frozen retinal tissue available (n = 18), the immunoblot was positive for PrPres. Two out of the 51 sCJD showed the deposition of PrPres also in the optic nerve, corresponding to an immunostaining delineating stellate cells and associated with the presence of numerous CD68- and CD45-positive cells. Our results demonstrate the presence of the pathological form of prion protein not only in the retina of all sCJD cases analysed, but also in optic nerve in a small subset of sCJD patients, a finding previously described only in variant CJD and in experimental animal models. Moreover, our data suggest a correlation between the deposition of PrPres and inflammatory changes in the optic nerve in sCJD.
AD M. Mangierie (mmangieri@istituto-besta.it), G.Giaccone, L. Limido, G. Di Fede, S. Suardi, R. Capobianco, O. Buginai, F. Tagliavini: Istituto Nazionale Neurologico Carlo Besta, Division of Neuropathology and Neurology 5, Milano, Italy; P. Fociani: Ospedale Luigi Sacco, Division of Pathology, Università di Milano, Milano, Italy
SP englisch
PO Italien