title: Chronic wasting disease prions are not transmissible to transgenic mice overexpressing human prion protein.
abstract: Chronic wasting disease (CWD) is a prion disease that affects free-ranging and captive cervids, including mule deer, white-tailed deer, Rocky Mountain elk and moose. CWD-infected cervids have been reported in 14 USA states, two Canadian provinces and in South Korea. The possibility of a zoonotic transmission of CWD prions via diet is of particular concern in North America where hunting of cervids is a popular sport. To investigate the potential public health risks posed by CWD prions, we have investigated whether intracerebral inoculation of brain and spinal cord from CWD-infected mule deer transmits prion infection to transgenic mice overexpressing human prion protein with methionine or valine at polymorphic residue 129. These transgenic mice have been utilized in extensive transmission studies of human and animal prion disease and are susceptible to BSE and vCJD prions, allowing comparison with CWD. Here, we show that these mice proved entirely resistant to infection with mule deer CWD prions arguing that the transmission barrier associated with this prion strain/host combination is greater than that observed with classical BSE prions. However, it is possible that CWD may be caused by multiple prion strains. Further studies will be required to evaluate the transmission properties of distinct cervid prion strains as they are characterized.
authors.affiliation: MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK.
authors.forename: Malin K
authors.initials: MK
authors.lastname: Sandberg
authors.forename: Huda
authors.initials: H
authors.lastname: Al-Doujaily
authors.forename: Christina J
authors.initials: CJ
authors.lastname: Sigurdson
authors.forename: Markus
authors.initials: M
authors.lastname: Glatzel
authors.forename: Catherine
authors.initials: C
authors.lastname: O'Malley
authors.forename: Caroline
authors.initials: C
authors.lastname: Powell
authors.forename: Emmanuel A
authors.initials: EA
authors.lastname: Asante
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Linehan
authors.forename: Sebastian
authors.initials: S
authors.lastname: Brandner
authors.forename: Jonathan D F
authors.initials: JD
authors.lastname: Wadsworth
authors.forename: John
authors.initials: J
authors.lastname: Collinge
mesh_terms: D000818:Animals; D000835:Animals, Wild; D004195:Disease Models, Animal; D006801:Humans; D007113:Immunity, Innate; D051379:Mice; D008822:Mice, Transgenic; D011328:Prions / Q000096:biosynthesis* / Q000235:genetics; D012418:Ruminants; D013045:Species Specificity; D034081:Wasting Disease, Chronic / Q000635:transmission*; D015047:Zoonoses
chemical_list: D011328:Prions