NR AXKC
AU Cali,I.; Parchi,P.; Langeveld,J.P.M.; Zou,W.Q.; Gambetti,P.
TI Coexistence of Both PrPsc Type 1 and 2 in sCJD: Does it Affect the Phenotype?
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Pathology and Pathogenesis P03.179
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB
In sporadic Creutzfeldt-Jakob disease (sCJD) five phenotypically distinct subtypes have been identified based on the methionine (M)/valine (V) polymorphic genotype of codon 129 and two PK-resistant scrapie prion protein (PrPsc) types, which migrate in gel to either 21 kDa (PrPsc type 1) or 19 kDa (PrPsc type 2). sCJD is characterized by phenotypic heterogeneity. The co-existence of both PrPsc types has recently been reported and may complicate the diagnosis.
In the present study we analyze the distribution of the two PrPsc types in various brain areas as well as the PrPsc resistance to PK digestion using a rigorous procedure according to Notari et al [Human PrPsc "Typing" pitfalls associated with the use of type 1 selective antibodies combined with relative inefficient hydrolysis of PrPsc by proteinase K, poster presentation, NeuroPrion 2006, Torino], with the intent of assessing the co-occurrence of fully PK-resistant PrPsc types 1 and 2 in a ratio of
50:50 or 60:40 while ascertaining that intermediate fragments are completely digested. We are studying the effect of the co-existence of the two PrPsc types, which occurs in either the same or different brain regions, on the sCJD disease phenotype by examining clinical history and neuropathological changes. (Supported by NIH AG-14359 and CDC UR8/CCU515004 awards, and the Charles S. Britton Fund)
AD I. Cali, W.Q. Zou, P. Gambetti, Case Western Reserve University, Pathology, USA; P. Parchi, Universita' di Bologna, Scienze Neurologiche, Italy; J. Langeveld, Central Institute for Animal Disease Control, Netherlands
SP englisch
PO Schottland