Chronic wasting disease: Emerging prions and their potential risk
Bibliographic record
Abstract
Prions cause fatal neurodegenerative diseases in humans and animals by converting the cellular prion protein PrP C into aggregation-prone PrP Sc .Chronic wasting disease (CWD) is a prion disease or transmissible spongiform encephalopathy (TSE) of free-ranging and farmed cervids.CWD is highly contagious and transmitted through horizontal transmission enabled by the shedding of prions in excreta and their persistence in the environment.The disease is undergoing a dramatic spread across North America, has been found in South Korea, and, recently, has been identified for the first time in Europe in free-ranging reindeer (Rangifer tarandus tarandus) and moose in Norway. CWDCWD first appeared in North America in captive animals in 1969 when biologists conducting a physiological study recognized that their animals often died with a syndrome of weight loss and behavioral changes.The biologists thought that the animals were probably dying of a nutritional deficiency due to toxin exposure or stressful conditions of captivity.In 1978, histological examination of brains of affected deer from wildlife research facilities in Colorado and Wyoming, United States, allowed us, for the first time, to recognize TSE features and define CWD as such [1].Since then, CWD has had an impressive expansion and a very rapid spread in North America; the geographic range of CWD includes 21 US states and 2 provinces in Canada (Saskatchewan and Alberta) and is most likely to continue to grow [2].Disease prevalence in free-ranging deer can be as high as 40% in the most endemic areas in Colorado and Wyoming [3].More recently, CWD has been described in Northern Europe [4].CWD is a slowly developing disease, with prolonged incubation periods.Experimental inoculation of mule deer has confirmed a 2-to 4-year incubation time [3].The disease has been found in yearling deer and elk as well as in adult animals as old as 15 years.Clinical symptoms include physical and behavioral changes [5].All these symptoms can be subtle early in the disease or fall within the normal repertoire of behavior or seasonal body mass fluctuations.Therefore, diagnosis based on clinical signs is not reliable and pathological or biochemical analyses of brain or lymphatic tissue are necessary.These will reveal neuronal vacuolation and spongiform changes, astrocytosis, and PrP Sc accumulation in the brain or PrP Sc deposits in lymphoid tissues.In most prion diseases, PrP Sc and infectivity are restricted mainly to the brain.However, in CWD, PrP Sc can be found in many extraneural tissues, body fluids, and excreta, facilitating horizontal transmission.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.002 | 0.002 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.001 | 0.002 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.001 | 0.002 |
| Insufficient payload (model declined to judge) | 0.004 | 0.002 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".