NR ATOC
AU Suardi,S.; Mangieri,M.; Limido,L.; Di Fede,G.; Capobianco,R.; van Zijderveld,F.G.; Langeveld,J.P.M.; Giaccone,G.; Tagliavini,F.
TI A subpopulation of type 1 PpPres is present in all CJD patients with type 2 PrPres
QU International Conference - Prion 2005: Between fundamentals and society's needs - 19.10.-21.10.2005, Congress Center Düsseldorf - Poster Session: Human prions, risk of blood products, and therapy HUMAN-26
PT Konferenz-Poster
AB In sporadic Creutzfeldt-Jakob disease (sCJD) two major types of PrPres (i.e., type 1 and type 2) have been recognized based on the electrophoretic mobility of the protease-resistant core. Previous studies showed that both PrPres types coexist in the cerebral cortex and/or subcortical gray structures of 25-30% of sCJD patients, although the extent and brain regional distribution of this event varies from case to case. We analyzed samples of cerebral cortex, subcortical grey structures and cerebellum of a series of sCJD patients (n=50) using the new monoclonal antibody 12B2, that specifically recognizes an epitope corresponding to residues W89-G93 of human PrP. The patients selected for the study had only type 1 (n=20) or type 2 (n=30) PrPres in all brain regions examined, when Western blot analysis was performed with the antibody 3F4. After proteinase K digestion, 12B2 reacted selectively with type 1 PrPres while it did not label type 2 PrPres. Surprisingly, 12B2 revealed the presence of a population of type 1 PrPres in all patients classified as "pure" type 2 CJD cases with the antibody 3F4. The amount of type 1 PrPres varied between patients and among different regions of the same brain, and was not influenced by the codon 129 PRNP genotype. A similar finding was obtained with brain tissue of a patient with variant Creutzfeldt-Jakob disease. These results indicate that occurence of variable amount of a population of type 1 PrPres is the rule in all CJD patients. The absence of reactivity of this population with the antibody 3F4 suggests that its folded state might differ from that of the typical type 1 PrPres.
AD Silvia Suardi, Michela Mangieri, Lucia Limido, Giuseppe Di Fede, Raffaella Capobianco, Giorgio Giaccone, Fabrizio Tagliavini, Istituto Nazionale Neurologico "C. Besta", Milano, Italy; Fred van Zijderveld, Jan Langeveld, CIDC-Lelystad, Lelystad, The Netherlands
SP englisch
PO Deutschland