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Title: CHRONIC WASTING DISEASE OF ELK: TRANSMISSIBILITY TO HUMANS EXAMINED BY TRANSGENIC MOUSE MODELS

Author
item KONG, QINGZHONG - CASE WESTERN RESERVE UNIV
item HUANG, SHENGHAI - CASE WESTERN RESERVE UNIV
item ZOU, WENQUAN - CASE WESTERN RESERVE UNIV
item VANEGAS, DIFERNANDO - CASE WESTERN RESERVE UNIV
item WANG, MEILING - CASE WESTERN RESERVE UNIV
item WU, DI - CASE WESTERN RESERVE UNIV
item YUAN, JUE - CASE WESTERN RESERVE UNIV
item ZHENG, MENGJIE - CASE WESTERN RESERVE UNIV
item BAI, HUA - CASE WESTERN RESERVE UNIV
item DENG, HUAYUN - CASE WESTERN RESERVE UNIV
item CHEN, KEN - ALBERT EINSTEIN COL OF ME
item Allen, Jenny
item O'Rourke, Katherine
item BELAY, ERMIAS - NCID-CDCP
item SCHONBERGER, LAWRENCE - NCID-CDCP
item PETERSEN, ROBERT - CASE WESTERN RESERVE UNIV
item SY, MAN-SUN - CASE WESTERN RESERVE UNIV
item CHEN, SHU - CASE WESTERN RESERVE UNIV
item GAMBETTI, PIERLUIGI - CASE WESTERN RESERVE UNIV

Submitted to: Journal of Neuroscience
Publication Type: Peer Reviewed Journal
Publication Acceptance Date: 7/19/2005
Publication Date: 8/31/2005
Citation: Kong, Q., Huang, S., Zou, W., Vanegas, D., Wang, M., Wu, D., Yuan, J., Zheng, M., Bai, H., Deng, H., Chen, K., Allen, J., O'Rourke, K.I., Belay, E.D., Schonberger, L.B., Petersen, R.B., Sy, M., Chen, S.G., Gambetti, P. 2005. Chronic Wasting Disease of Elk: Transmissibility to Humans Examined by Transgenic Mouse Models. The Journal of Neuroscience. 25(35):7944-7949.

Interpretive Summary: Chronic wasting disease is a fatal, transmissible prion disorder of deer and elk. The prion diseases are associated with interaction between the abnormal prion protein molecules from the diseased animals and the normal prion proteins produced by healthy animals. The potential for transmission of CWD to humans having contact with infected deer or elk is not known. In this study, the problem was addressed through the use of transgenic mice, in which the gene for the normal mouse prion protein was replaced by the gene for the elk or the human prion protein precursor. These transgenic mice were inoculated with brain homogenates from elk with CWD. The transgenic mice with the elk prion gene developed disease in approximately 4 months. The transgenic mice with the human prion gene showed no evidence of infection at 14 to 17 months, close to the lifespan of the mouse. These findings indicate that differences in the prion genetics between humans and elk may limit the risk of transmission.

Technical Abstract: Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy of cervids (deer/elk) in the United States and Canada. The potential risk of CWD transmission to humans is not known. In this report, brain homogenates fromelk with CWD were inoculated into transgenic mice expressing the human or elk prion protein. The cervidized transgenic mice became infected after approximately 4 months. No evidence of a transmssible spongiform encephalopathy were detected in mice with the human transgene at 14 to 17 months after inoculation. These data indicate that there is a substantial species barrier for transmission of elk CWD to humans.