New model for chronic wasting disease

They present these findings in the June 2010 issue of The American Journal of Pathology.

Chronic wasting disease is a fatal prion-induced disease, similar to mad cow disease, that affects cervids such as deer, elk, and moose. It is a neurodegenerative disease typified by chronic weight-loss leading to death. Prions are infectious agents composed primarily of proteins that are thought to be propagated by transmitting a mis-folded protein state. Due to the lack of an appropriate small animal model, little is known about cervid chronic wasting disease.

Using a mouse model of chronic wasting disease that expresses cervid prion protein (PrP), Seelig et al examined the susceptibility, pathogenesis, and transmission of cervid chronic wasting disease. They found that cervid PrP^C (protease-sensitive PrP) was expressed in a number of different tissues, including lymphoid, nervous, hematopoietic, endocrine, and certain epithelial tissues, in this model. Additionally, disease could be transferred by various infectious methods, including injection into the brain, blood stream, and gut. It could also be transmitted orally, although the oral route required a larger infecting dose. Furthermore, this disease could be transferred between animals without experimental intervention to uninfected mice, highlighting the suitability of this system in studying cervid transmissible spongiform encephalopathy.

Dr. Hoover's group suggests that "cervidized transgenic mice substantially recapitulate the clinical, neuropathologic, and PrP^RES tropism and transmission patterns reported in the native cervid species and [that] studies in Tg[CerPrP] mice can provide additional insights into the trafficking, shedding, and lateral transmission of [chronic wasting disease] prions."