Large animal models for chronic wasting disease

Abstract

Chronic wasting disease (CWD) is a fatal neurodegenerative prion disease of cervid species including deer, elk, moose and reindeer. The disease has shown both geographic and species expansion since its discovery in the late 1960’s and is now recognized in captive and free-ranging cervid populations in North America, Asia and Europe. The facile transmission of CWD is unique among prion diseases and has resulted in growing concern for cervid populations and human public health. The development of native cervid host models with longitudinal monitoring has revealed new insights about CWD pathogenesis and transmission dynamics. More than 20 years of experimental studies conducted in these models, using biologically relevant routes of infection, have led to better understanding of many aspect of CWD infections. This review addresses some of these insights, including: (i) the temporal intra-host trafficking of CWD prions in tissues and bodily fluids, (ii) the presence of infectivity shed in bodily excretions that may help explain the facile transmission of CWD, (iii) mother-to-offspring CWD transmission, (iv) the influence of some Prnp polymorphisms on CWD susceptibility, and (vi) continued development of vaccine strategies to mitigate CWD.

Vaccines for prion diseases: a realistic goal?

“…For experimental studies in cervids, there are two basic scenarios. One is controlled oral challenge studies in cervid species kept inside, for example, WTD and reindeer (Mathiason et al 2006 ; Mathiason 2022 ; Mitchell et al 2012 ). Similar studies have been done with outdoor-held captive elk in appropriate confinements (Basu et al 2012 ).…”

New developments in prion disease research

“…Large animal models are used to study bovine, sheep, and cervid prions in the original host setting. The chapter by Candace Mathiason describes native cervid host models, and how they can be used for longitudinal monitoring of prion diseases parameters, providing valuable insights into CWD pathogenesis and transmission (Mathiason 2022). Whereas such complex studies can be performed only in selected facilities, the widely used in vivo work horse of prion research are rodent models.…”