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Chronic Wasting Disease

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Prion Proteins

Part of the book series: Topics in Current Chemistry ((TOPCURRCHEM,volume 305))

Abstract

Chronic wasting disease (CWD) is a prion disease of free-ranging and farmed ungulates (deer, elk, and moose) in North America and South Korea. First described by the late E.S. Williams and colleagues in northern Colorado and southern Wyoming in the 1970s, CWD has increased tremendously both in numerical and geographical distribution, reaching prevalence rates as high as 50% in free-ranging and >90% in captive deer herds in certain areas of USA and Canada. CWD is certainly the most contagious prion infection, with significant horizontal transmission of infectious prions by, e.g., urine, feces, and saliva. Dissemination and persistence of infectivity in the environment combined with the appearance in wild-living and migrating animals make CWD presently uncontrollable, and pose extreme challenges to wild-life disease management. Whereas CWD is extremely transmissible among cervids, its trans-species transmission seems to be restricted, although the possible involvement of rodent and carnivore species in environmental transmission has not been fully evaluated. Whether or not CWD has zoonotic potential as had Bovine spongiform encephalopathy (BSE) has yet to be answered. Of note, variant Creutzfeldt–Jakob disease (vCJD) was only detected because clinical presentation and age of patients were significantly different from classical CJD. Along with further understanding of the molecular biology and pathology of CWD, its transmissibility and species restrictions and development of methods for preclinical diagnosis and intervention will be crucial for effective containment of this highly contagious prion disease.

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Authors and Affiliations

  1. Department of Veterinary Sciences, University of Wyoming, 1174 Snowy Range Road, Laramie, WY, 82070, USA

    Sabine Gilch, Nandini Chitoor, Yuzuru Taguchi, Melissa Stuart, Jean E. Jewell & Hermann M. Schätzl

  2. Department of Molecular Biology, University of Wyoming, 1000 E. University Ave., Laramie, WY, 82071, USA

    Sabine Gilch, Nandini Chitoor, Yuzuru Taguchi, Melissa Stuart, Jean E. Jewell & Hermann M. Schätzl

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  2. Nandini Chitoor
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  3. Yuzuru Taguchi
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  4. Melissa Stuart
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  5. Jean E. Jewell
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  6. Hermann M. Schätzl
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Corresponding author

Correspondence to Hermann M. Schätzl .

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  1. Adolf-Butenandt-Institute, Abt. Biochemie, Universität München, Schillerstr. 44, München, 80336, Germany

    Jörg Tatzelt

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© 2011 Springer-Verlag Berlin Heidelberg

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Gilch, S., Chitoor, N., Taguchi, Y., Stuart, M., Jewell, J.E., Schätzl, H.M. (2011). Chronic Wasting Disease. In: Tatzelt, J. (eds) Prion Proteins. Topics in Current Chemistry, vol 305. Springer, Berlin, Heidelberg. https://doi.org/10.1007/128_2011_159

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