Spongiform Encephalopathy of Rocky Mountain Elk

Williams, Elizabeth; Young, Stuart

doi:10.7589/0090-3558-18.4.465

Cited by 251 publications

(145 citation statements)

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“…Therefore, the capacity for CWD to cross species barriers has been of great interest. Until recently, natural CWD infections were believed to infect only white-tailed deer, mule deer, and Rocky Mountain elk [89,90,92]. This ended in 2006, when a report indicated that Shira's moose could be orally-infected with brain homogenate from CWD-infected mule deer, and shortly thereafter a natural case of a CWD-infected moose was discovered [4,42].…”

Section: Natural Cwd Infectionsmentioning

confidence: 99%

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A prion disease of cervids: Chronic wasting disease

2008

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-Chronic wasting disease (CWD) is a prion disease of deer, elk, and moose, initially recognized in Colorado mule deer. The discovery of CWD beyond the borders of Colorado and Wyoming, in Canada and as far east as New York, has led to its emergence as a prion disease of international importance. Epidemiological studies indicate that CWD is horizontally transmitted among free-ranging animals, potentially indirectly by prion-containing secreta or excreta contaminating the environment. Experimental CWD transmission attempts to other wild and domestic mammals and to transgenic mice expressing the prion protein of cattle, sheep, and humans have shed light on CWD species barriers. Transgenic mice expressing the cervid prion protein have proven useful for assessing the genetic influences of Prnp polymorphisms on CWD susceptibility. Accumulating evidence of CWD pathogenesis indicates that the misfolded prion protein or prion infectivity seems to be widely disseminated in many nonneural organs and in blood. This review highlights contemporary research findings in this prion disease of free-ranging wildlife.

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Section: Natural Cwd Infectionsmentioning

confidence: 99%

“…In 1978, CWD was first recognized as a new TSE of captive mule deer in Colorado by pathologists Williams and Young [89]. Soon thereafter, a prion disease was reported in elk in Wyoming [90]. CWD was believed to be limited to this central region of the US for two decades.…”

Section: Introductionmentioning

confidence: 99%

A prion disease of cervids: Chronic wasting disease

2008

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“…CWD was first detected in cervids in the late 1960s in Colorado [2,3] and has since spread to an increasing number of other US states and Canadian provinces [4,5]. The disease is transmitted horizontally by contact with pathogenic prions shed in bodily fluids [6,7] and vertically from mother to offspring [8,9].…”

Section: Introductionmentioning

confidence: 99%

Influence of the geographic distribution of prion protein gene sequence variation on patterns of chronic wasting disease spread in white-tailed deer (Odocoileus virginianus)

Brandt

Green

Ishida

et al. 2018

Prion

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Managing and controlling the spread of diseases in wild animal populations is challenging, especially for social and mobile species. Effective management benefits from information about disease susceptibility, allowing limited resources to be focused on areas or populations with a higher risk of infection. Chronic wasting disease (CWD), a transmissible spongiform encephalopathy that affects cervids, was detected in Colorado in the late 1960s. CWD was detected in Illinois and Wisconsin in 2002 and has since spread through many counties. Specific nucleotide variations in the prion protein gene (PRNP) sequence have been associated with reduced susceptibility to CWD in white-tailed deer. Though genetic resistance is incomplete, the frequency of deer possessing these mutations in a population is an important factor in disease spread (i.e. herd immunity). In this study we sequenced 625 bp of the PRNP gene from a sampling of 2433 deer from Illinois and Wisconsin. In north-central Illinois where CWD was first detected, counties had a low frequency of protective haplotypes (frequency <0.20); whereas in northwestern Illinois counties, where CWD cases have only more recently been detected, the frequency of protective haplotypes (frequency >0.30) was much higher (p < 0.05). Protective haplotype frequencies varied significantly among infected and uninfected geographic areas. The frequency of protective PRNP haplotypes may contribute to population level susceptibility and may shape the way CWD has spread through Illinois. Analysis of PRNP haplotype distribution could be a useful tool to assess CWD risk and allocate resources to contain and reduce the spread of infection.

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“…13 Similar diseases have been described in many other species, including mink, 19 deer, 36 elk, 37 cattle, 35 cats, 38 zoo ruminants, 7 and humans. 3 The primary tissue affected is the central nervous system, resulting in a variety of clinical neurologic signs.…”

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confidence: 96%

Immunohistochemical Detection of Prion Protein in Sheep with Scrapie

et al. 1993

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Abstract. Prion protein (PrP), which is involved in the pathogenesis of scrapie, occurs in 2 forms. The form extracted from scrapie brain is protease resistant (PrP-res), whereas PrP from normal brain is protease sensitive (PrP-sen). This study examined whether PrP-res could be detected in brains of sheep with scrapie by immunohistochemistry (IHC). A suitable IHC procedure was developed using brain tissue from hamsters that had been inoculated with the transmissible mink encephalopathy agent. Tissue samples were fixed in PLP (periodate, lysine, paraformaldehyde) that contained paraformaldehyde at a concentration of 0.125%. Before application of the IHC technique, tissue sections were deparaffinized and treated with formic acid to simultaneously enhance PrP-res immunoreactivity and degrade PrP-sen. Primary antibody was obtained from a rabbit immunized to PrP-res extracted from brains of mice with experimentally induced scrapie. Brains from 21 sheep with histopathologically confirmed scrapie were examined by IHC. In all 21 brains, PrP-res was widely distributed throughout the brain stem. Staining was particularly intense in neuronal cell bodies and around blood vessels. The IHC technique successfully detected PrP-res in brain samples that had been frozen or that were severely autolyzed before fixation in PLP. Brains from 11 scrapie-suspect sheep that were not considered histologically positive were also examined by IHC. PrP-res was found in 4 of these brains. Sections of brains from 14 clinically normal sheep did not have detectable PrP-res. Results of this study indicate that IHC detection of PrP-res is equivalent, and perhaps superior, to histopathology for the diagnosis of scrapie in sheep. Furthermore, IHC is applicable to tissues that have autolytic changes or processing artifacts that prevent satisfactory histopathologic evaluation for lesions of scrapie.

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Spongiform Encephalopathy of Rocky Mountain Elk

Cited by 251 publications

References 0 publications

A prion disease of cervids: Chronic wasting disease

A prion disease of cervids: Chronic wasting disease

Influence of the geographic distribution of prion protein gene sequence variation on patterns of chronic wasting disease spread in white-tailed deer (Odocoileus virginianus)

Immunohistochemical Detection of Prion Protein in Sheep with Scrapie

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