NR AWFE
AU Gambetti,P.
TI Prion strains in human prion diseases
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Oral sessions ORAL-25
PT Konferenz-Vortrag
AB The prion protein (PrP) genotype, as determined by the polymorphic codon 129, is thought to have an effect on the conformational characteristics of the scrapie PrP (PrPsc) revealed by the size of the protease-resistant PrPsc fragment or PrPsc type. Based on PrP genotype and PrPsc type we have proposed a classification of sporadic Creutzfeldt-Jakob disease (sCJD) in six subtypes. Each of these subtypes appears to be associated with a distinct PrPsc or prion strain. The purpose of this presentation is to review the current state of human prion strains, of the disease phenotypes with which the prion strains associate, and of current classifications of human prion diseases. Alternative classifications proposed by others or based on our own experience in examining over thousand cases of prion disease will be discussed. Of special interest is the recent identification of a novel phenotype of human prion disease associated with a distinct PrPsc strain that is largely protease sensitive. The contribution of transgenic mice expressing human PrPc to the study of human prion strains and disease phenotypes will also be examined. (Supported by NIH AG-14359 and CDC UR8/CCU515004 awards and the Charles S. Britton Fund).
AD National Prion Disease Pathology Surveillance Center, Department of Pathology, Case Western Reserve University, Cleveland, USA. E-mail: pxg13@case.edu
SP englisch
PO Italien