title: BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein.
abstract: 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 PrP(Sc) 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.
authors.affiliation: MRC Prion Unit, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK.
authors.forename: Emmanuel A
authors.initials: EA
authors.lastname: Asante
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Linehan
authors.forename: Melanie
authors.initials: M
authors.lastname: Desbruslais
authors.forename: Susan
authors.initials: S
authors.lastname: Joiner
authors.forename: Ian
authors.initials: I
authors.lastname: Gowland
authors.forename: Andrew L
authors.initials: AL
authors.lastname: Wood
authors.forename: Julie
authors.initials: J
authors.lastname: Welch
authors.forename: Andrew F
authors.initials: AF
authors.lastname: Hill
authors.forename: Sarah E
authors.initials: SE
authors.lastname: Lloyd
authors.forename: Jonathan D F
authors.initials: JD
authors.lastname: Wadsworth
authors.forename: John
authors.initials: J
authors.lastname: Collinge
mesh_terms: D000818:Animals; D002417:Cattle; D003062:Codon / Q000235:genetics; D007562:Creutzfeldt-Jakob Syndrome / Q000378:metabolism*; D016643:Encephalopathy, Bovine Spongiform / Q000378:metabolism*; D006801:Humans; D007150:Immunohistochemistry; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D011110:Polymorphism, Genetic; D011328:Prions / Q000008:administration & dosage / Q000235:genetics* / Q000378:metabolism*
chemical_list: D003062:Codon; D011328:Prions