Abstract
The horizontal transmission of prion diseases has been well characterized in bovine spongiform encephalopathy (BSE), chronic wasting disease (CWD) of deer and elk and scrapie of sheep, and has been regarded as the primary mode of transmission. Few studies have monitored the possibility of vertical transmission occurring within an infected mother during pregnancy. To study the potential for and pathway of vertical transmission of CWD in the native cervid species, we used a small cervid model–the polyestrous breeding, indoor maintainable, Reeves’ muntjac deer–and determined that the susceptibility and pathogenesis of CWD in these deer reproduce that in native mule and white-tailed deer. Moreover, we demonstrate here that CWD prions are transmitted from doe to fawn. Maternal CWD infection also appears to result in lower percentage of live birth offspring. In addition, evolving evidence from protein misfolding cyclic amplification (PMCA) assays on fetal tissues suggest that covert prion infection occurs in utero. Overall, our findings demonstrate that transmission of prions from mother to offspring can occur, and may be underestimated for all prion diseases.
Highlights
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative diseases that affect a variety of species, including humans (Kuru; variant Creutzfeldt-Jakob disease [variant CJD (vCJD)]), cattle, sheep, mink, domestic and nondomestic cats and cervids
It is clear that TSEs are spread by horizontal means of transmission via oral ingestion (Kuru, bovine spongiform encephalopathy (BSE), vCJD, TME, FSE, chronic wasting disease (CWD)) and environmental contact with infectious prions, as well as by medical means including blood transfusion, or iatrogenic exposure
It is known that vCJD can be transmitted from human to human via blood transfusion from both symptomatic and asymptomatic donors [3,4,5,6] and that a prolonged asymptomatic incubation period occurs in 129 Met/
Summary
Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative diseases that affect a variety of species, including humans (Kuru; variant Creutzfeldt-Jakob disease [vCJD]), cattle (bovine spongiform encephalopathy [BSE]), sheep (scrapie), mink (transmissible mink encephalopathy [TME]), domestic and nondomestic cats (feline spongiform encephalopathy [FSE]) and cervids (chronic wasting disease [CWD]).The transmission of prions, while remaining poorly understood, has obvious public health significance and requires sustained reappraisal of present concepts for both TSE spread and intervention strategies. Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative diseases that affect a variety of species, including humans (Kuru; variant Creutzfeldt-Jakob disease [vCJD]), cattle (bovine spongiform encephalopathy [BSE]), sheep (scrapie), mink (transmissible mink encephalopathy [TME]), domestic and nondomestic cats (feline spongiform encephalopathy [FSE]) and cervids (chronic wasting disease [CWD]). It is clear that TSEs are spread by horizontal means of transmission via oral ingestion (Kuru, BSE, vCJD, TME, FSE, CWD) and environmental (scrapie, CWD) contact with infectious prions, as well as by medical means including blood transfusion (vCJD, CWD, scrapie), or iatrogenic exposure (vCJD, sCJD). A variant form of the human prion disease CJD, known as variant CJD (vCJD), was attributed to the ingestion of BSEinfected meat products [1,2]. It is known that vCJD can be transmitted from human to human via blood transfusion from both symptomatic and asymptomatic donors [3,4,5,6] and that a prolonged asymptomatic incubation period occurs in 129 Met/
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
