scholarly journals Detection of the Abnormal Isoform of the Prion Protein Associated With Chronic Wasting Disease in the Optic Pathways of the Brain and Retina of Rocky Mountain Elk (Cervus elaphus nelsoni)

2010 ◽  
Vol 47 (3) ◽  
pp. 536-546 ◽  
Author(s):  
T. R. Spraker ◽  
K. I. O’Rourke ◽  
T. Gidlewski ◽  
J. G. Powers ◽  
J. J. Greenlee ◽  
...  
2015 ◽  
Vol 27 (4) ◽  
pp. 431-441 ◽  
Author(s):  
Terry R. Spraker ◽  
Thomas Gidlewski ◽  
Jenny G. Powers ◽  
Tracy Nichols ◽  
Aru Balachandran ◽  
...  

The purpose of our study was to describe the progressive accumulation of the abnormal conformer of the prion protein (PrPCWD) and spongiform degeneration in a single section of brain stem in Rocky Mountain elk ( Cervus elaphus nelsoni) with chronic wasting disease (CWD). A section of obex from 85 CWD-positive elk was scored using the presence and abundance of PrPCWD immunoreactivity and spongiform degeneration in 10 nuclear regions and the presence and abundance of PrPCWD in 10 axonal tracts, the subependymal area of the fourth ventricle, and the thin subpial astrocytic layer (glial limitans). Data was placed in a formula to generate an overall obex score. Data suggests that PrPCWD immunoreactivity and spongiform degeneration has a unique and relatively consistent pattern of progression throughout a section of obex. This scoring technique utilizing a single section of obex may prove useful in future work for estimating the presence and abundance of PrPCWD in peripheral tissues and the nervous system in elk with CWD.


2006 ◽  
Vol 18 (6) ◽  
pp. 553-557 ◽  
Author(s):  
Terry R. Spraker ◽  
Thomas L. Gidlewski ◽  
Aru Balachandran ◽  
Kurt C. VerCauteren ◽  
Lynn Creekmore ◽  
...  

1999 ◽  
Vol 80 (10) ◽  
pp. 2765-2679 ◽  
Author(s):  
K. I. O’Rourke ◽  
T. E. Besser ◽  
M. W. Miller ◽  
T. F. Cline ◽  
T. R. Spraker ◽  
...  

The PrP gene encodes the putative causative agent of the transmissible spongiform encephalopathies (TSEs), a heterogeneous group of fatal, neurodegenerative disorders including human Creutzfeldt–Jakob disease, bovine spongiform encephalopathy, ovine scrapie and chronic wasting disease (CWD) of North American deer and elk. Polymorphisms in the PrP gene are associated with variations in relative susceptibility, pathological lesion patterns, incubation times and clinical course of TSEs of humans, mice and sheep. Sequence analysis of the PrP gene from Rocky Mountain elk showed only one amino acid change (Met to Leu at cervid codon 132). Homozygosity for Met at the corresponding polymorphic site (Met to Val) in humans (human codon 129) predisposes exposed individuals to some forms of Creutzfeldt–Jakob disease. In this study, Rocky Mountain elk homozygous for PrP codon 132 Met were over-represented in both free- ranging and farm-raised CWD-affected elk when compared to unaffected control groups.


2006 ◽  
Vol 87 (11) ◽  
pp. 3443-3450 ◽  
Author(s):  
Jean E. Jewell ◽  
Jeremy Brown ◽  
Terry Kreeger ◽  
Elizabeth S. Williams

To investigate the possible presence of disease-associated prion protein (PrPd) in striated muscle of chronic wasting disease (CWD)-affected cervids, samples of diaphragm, tongue, heart and three appendicular skeletal muscles from mule deer (Odocoileus hemionus), white-tailed deer (Odocoileus virginianus), elk (Cervus elaphus nelsoni) and moose (Alces alces shirasi) were examined by ELISA, Western immunoblot and immunohistochemistry (IHC). PrPd was detected in samples of heart muscle from seven of 16 CWD-infected white-tailed deer, including one free-ranging deer, and in 12 of 17 CWD-infected elk, but not in any of 13 mule deer samples, nor in the single CWD-infected moose. For white-tailed deer, PrPd was detected by Western blot at multiple sites throughout the heart; IHC results on ventricular sections of both elk and white-tailed deer showed positive staining in cardiac myocytes, but not in conduction tissues or nerve ganglia. Levels of PrPd in cardiac tissues were estimated from Western blot band intensity to be lower than levels found in brain tissue. PrPd was not detected in diaphragm, triceps brachii, semitendinosus, latissiumus dorsi or tongue muscles for any of the study subjects. This is the first report of PrPd in cardiac tissue from transmissible spongiform encephalopathy-infected ruminants in the human food chain and the first demonstration by immunological assays of PrPd in any striated muscle of CWD-infected cervids.


2006 ◽  
Vol 87 (12) ◽  
pp. 3773-3780 ◽  
Author(s):  
Giuseppe LaFauci ◽  
Richard I. Carp ◽  
Harry C. Meeker ◽  
Xuemin Ye ◽  
Jae I. Kim ◽  
...  

Chronic wasting disease (CWD) of elk (Cervus elaphus nelsoni) and mule deer (Odocoileus hemionus) is one of three naturally occurring forms of prion disease, the others being Creutzfeldt–Jakob disease in humans and scrapie in sheep. In the last few decades, CWD has spread among captive and free-ranging cervids in 13 US states, two Canadian provinces and recently in Korea. The origin of the CWD agent(s) in cervids is not known. This study describes the development of a transgenic mouse line (TgElk) homozygous for a transgene array encoding the elk prion protein (PrPC) and its use in propagating and simulating CWD in mice. Intracerebral injection of one mule deer and three elk CWD isolates into TgElk mice led to disease with incubation periods of 127 and 95 days, respectively. Upon secondary passage, the incubation time was reduced to 108 and 90 days, respectively. Upon passage into TgElk mice, CWD prions (PrPSc) maintained the characteristic Western blot profiles seen in CWD-affected mule deer and elk and produced histopathological modifications consistent with those observed in the natural disease. The short incubation time observed on passage from cervid to mouse with both mule deer and elk CWD brain homogenates and the demonstrated capacity of the animals to propagate (mouse to mouse) CWD agents make the TgElk line a valuable model to study CWD agents in cervid populations. In addition, these results with this new transgenic line suggest the intriguing hypothesis that there could be more than one strain of CWD agent in cervids.


2013 ◽  
Vol 49 (2) ◽  
pp. 270-278 ◽  
Author(s):  
Ryan J. Monello ◽  
Jenny G. Powers ◽  
N. Thompson Hobbs ◽  
Terry R. Spraker ◽  
Katherine I. O’Rourke ◽  
...  

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