PLEASE NOTE HIGH INCIDENT OF SPORADIC CJD AND BSE IN THE ALAVA, VIZCAYA, AND GUIPUZCOA AREAS ? another coincident, or BSE related. remember, BSE will propagate as nvCJD and or sporadic CJD in humanized transgenic mice, and please NOTE ALSO, the close proximity of BASQUE COUNTRY SPAIN TO ALAVA, VIZCAYA, AND GUIPUZCOA AREAS ? SO, you have a high rate of sporadic CJD cases in an area of BSE and Nor-98 atypical scrapie cases, another spontaneous coincidence, or a related event ?
NOTE THE SPORADIC CJD INCIDENT RATE AT > 2 CASES PER MILLION ;
SEE CJD UPDATE 2010
SEE PAGE 4 ;
Notificaciones* al RNEETH (1993-febrero 2010)
• Por año diagnóstico. Existe 1 caso más sin año diagnóstico
The EMBO Journal (2002) 21, 6358 - 6366 doi:10.1093/emboj/cdf653
BSE prions propagate as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein
Emmanuel A. Asante1, Jacqueline M. Linehan1, Melanie Desbruslais1, Susan Joiner1, Ian Gowland1, Andrew L. Wood1, Julie Welch1, Andrew F. Hill1, Sarah E. Lloyd1, Jonathan D.F. Wadsworth1 and John Collinge1
1.MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College, Queen Square, London WC1N 3BG, UK Correspondence to John Collinge, E-mail: firstname.lastname@example.org
Received 1 August 2002; Accepted 17 October 2002; Revised 24 September 2002
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.