NR AOQN
AU Bishop,M.T.; Will,R.G.; Aitchison,L.; Baybutt,H.N.; Gall,E.; Hart,P.; Tuzi,N.L.; Manson,J.C.
TI Novel gene targeted transgenic mice as models for codon 129 disease association in 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 - BR-18
PT Konferenz-Poster
AB With the aim of modelling human TSE disease and studying the role of codon 129 in disease susceptibility in a mouse model, gene targeting was used as an approach to produce transgenic mice expressing the human prion protein gene. This allows for expression of transgenic prion protein mimicking that of the wildtype murine gene in both CNS and peripheral tissue. Level of expression can therefore be ruled out as effecting transmission studies. We have produced three lines of mice expressing the three codon 129 variants (Met/Met, Met/Val, and Val/Val). These lines were inbred to ensure any differences in transmission between the lines was due to the differences in the PrP genes and not to additional genetic background effects. The human transgenic prion protein has been shown to be: at levels similar to mouse prion protein in wildtype mice; Proteinase K sensitive; present in mono-, di-, and un-glycosylated forms; and attached to cellular membranes by a GPI anchor. The mice have been inoculated via intracerebral and intraperitoneal routes with vCJD(Genotype:MM, PrP isotype:2B), sCJD(MM1), sCJD(MV2A), sCJD(VV2A). The results will be presented.
AD M.T. Bishop, R.G. Will, National CJD Surveillance Unit, UK; M.T. Bishop, L. Aitchison, H. Baybutt, E. Gall, P. Hart, N. Tuzi, J.C. Manson, Institute for Animal Health, Neuropathogenesis Unit, UK
SP englisch
PO Deutschland