NR AAUB
AU Asante,E.A.; Linehan,J.M.; Desbruslais,M.; Joiner,S.; Gowland,I.; Wood,A.L.; Welch,J.; Hill,A.F.; Lloyd,S.E.; Wadsworth,J.D.F.; Collinge,J.
TI BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein
QU EMBO Journal 2002 Dec 2; 21(23): 6358-66
IA http://www.pubmedcentral.gov/articlerender.fcgi?tool=pubmed&pubmedid=12456643
PT journal article
AB Variant Creutzfeldt-Jakob disease (vCJD) has been recognized to date only in individuals homozygous for methionine at PRNP codon 129. Here we show that transgenic mice expressing human PrP methionine 129, inoculated with either bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the neuropathological and molecular phenotype of vCJD, consistent with these diseases being caused by the same prion strain. Surprisingly, however, BSE transmission to these transgenic mice, in addition to producing a vCJD-like phenotype, can also result in a distinct molecular phenotype that is indistinguishable from that of sporadic CJD with PrPsc type 2. These data suggest that more than one BSE-derived prion strain might infect humans; it is therefore possible that some patients with a phenotype consistent with sporadic CJD may have a disease arising from BSE exposure.
MH Animals; Cattle; Codon/genetics; Creutzfeldt-Jakob Syndrome/*metabolism; Encephalopathy, Bovine Spongiform/*metabolism; Humans; Immunohistochemistry; Mice; Mice, Inbred C57BL; Mice, Transgenic; Polymorphism, Genetic; Prions/administration & dosage/*genetics/*metabolism; Research Support, Non-U.S. Gov't
AD Dr. Emmanuel A. Asante, MRC Prion Unit & Neurogenetics Dept. Imperial College School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG, Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email: e.asante@prion.ucl.ac.uk; J.Collinge, MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK Corresponding author e-mail: j.collinge@prion.ucl.ac.uk
SP englisch
PO England
OR Prion-Krankheiten 1