NR AWOP
AU Tamgüney,G.; Giles,K.; Bosque,P.J.; Miller,M.W.; Safar,J.G.; DeArmond,S.J.; Prusiner,S.B.
TI Transmission of CWD to transgenic mice
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions PR-32
PT Konferenz-Poster
AB Chronic wasting disease (CWD) is a lethal prion disease in deer and elk. Distinctive among the prion diseases, it is transmitted among captive and free-ranging cervids. To study the biology of CWD prions, we generated five lines of transgenic (Tg) mice expressing prion protein (PrP) from Rocky Mountain elk (Cervus elaphus nelsoni), denoted Tg(ElkPrP), and two lines of Tg mice expressing PrP common to mule deer (Odocoileus hemionus) and white-tailed deer (Odocoileus virginianus), denoted Tg(DePrP). At 550 days of age none of the Tg(DePrP) or Tg(ElkPrP) mice exhibited spontaneous neurologic dysfunction. After inoculation, brain samples from CWD-positive elk, white-tailed deer, and mule deer produced disease in Tg(ElkPrP) mice between 180 and 200 days and in Tg(DePrP) mice between 300 and 400 days. Tg(MoPrP)4053 mice overexpressing wild-type mouse PrP-A were susceptible to one of eight cervid brain inocula in ~540 days. Brains of diseased mice revealed abundant PrP amyloid plaques upon neuropathologic analysis. Serial passaging of brain homogenates from symptomatic Tg(ElkPrP) mice produced disease in 120-190 days in Tg(ElkPrP) mice. Contrary to Tg(DePrP) and Tg(ElkPrP) mice, Tg mice overexpressing human, bovine, or ovine PrP did not develop prion disease after inoculation with CWD prions from among nine different isolates after >500 days. These results suggest that CWD prions from white-tailed deer, mule deer, and elk can readily transmit among these three cervid species.
AD G. Tamgüney, Institute for Neurodegenerative Diseases, University of California, San Francisco, California; K.Giles, P.J. Bosque, J. Safar: Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, California; S.J. DeArmond: Institute for Neurodegenerative Diseases, Department of Pathology, University of California, San Francisco, California; S.B. Prusiner: Institute for Neurodegenerative Diseases, Department of Neurology, Biochemistry and Biophysics, University of California, San Francisco, California; M.W. Miller: Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, Colorado. E-mail: etamguney@ind.ucsf.edu
SP englisch
PO Italien