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      Asymptomatic deer excrete infectious prions in feces

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          Abstract

          Infectious prion diseases 1 – scrapie of sheep 2 and chronic wasting disease (CWD) of several species in the deer family 3, 4 – are transmitted naturally within affected host populations. Although several possible sources of contagion have been identified in excretions and secretions from symptomatic animals 58 , the biological importance of these sources in sustaining epidemics remains unclear. Here we show that asymptomatic CWD-infected mule deer ( Odocoileus hemionus) excrete CWD prions in their feces long before they develop clinical signs of prion disease. Intracerebral (i.c.) inoculation of irradiated deer feces into transgenic (Tg) mice overexpressing cervid PrP revealed infectivity in 14 of 15 fecal samples collected from 5 deer at 7–11 months before the onset of neurological disease. Although prion concentrations in deer feces were considerably lower than in brain tissue from the same deer collected at the disease terminus, the estimated total infectious dose excreted in feces by an infected deer over the disease course may approximate the total contained in brain tissue. Prolonged fecal prion excretion by infected deer provides a plausible natural mechanism that might explain the high incidence and efficient horizontal transmission of CWD within deer herds 3, 4, 9 , as well as prion transmission between susceptible deer species.

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          Nobel Lecture: Prions

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            Environmental Sources of Prion Transmission in Mule Deer

            Whether transmission of the chronic wasting disease (CWD) prion among cervids requires direct interaction with infected animals has been unclear. We report that CWD can be transmitted to susceptible animals indirectly, from environments contaminated by excreta or decomposed carcasses. Under experimental conditions, mule deer (Odocoileus hemionus) became infected in two of three paddocks containing naturally infected deer, in two of three paddocks where infected deer carcasses had decomposed in situ ≈1.8 years earlier, and in one of three paddocks where infected deer had last resided 2.2 years earlier. Indirect transmission and environmental persistence of infectious prions will complicate efforts to control CWD and perhaps other animal prion diseases.
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              Chronic wasting disease of captive mule deer: a spongiform encephalopathy.

              In the past 12 years (1967-79) a syndrome we identify as chronic wasting disease has been observed in 53 mule deer (Odocoileus hemionus hemionus) and one black-tailed deer (Odocoileus hemionus columbianus) held in captivity in several wildlife facilities in Colorado and more recently in Wyoming. Clinical signs were seen in adult deer and included behavioral alterations, progressive weight loss and death in 2 weeks to 8 months. Gross necropsy findings included emaciation and excess rumen fluid admixed with sand and gravel. Consistent histopathologic change was limited to the central nervous system and characterized by widespread spongiform transformation of the neuropil, single of multiple intracytoplasmic vacuoles in neuronal perikaryons and intense astrocytic hypertrophy and hyperplasia. Presented is a clinical characterization of chronic wasting disease and pathologic evidence supporting the conclusion that the disease is a specific spontaneously occurring form of spongiform encephalopathy.
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                Author and article information

                Journal
                0410462
                6011
                Nature
                Nature
                0028-0836
                1476-4687
                7 September 2011
                9 September 2009
                24 September 2009
                4 October 2011
                : 461
                : 7263
                : 529-532
                Affiliations
                [1 ]Institute for Neurodegenerative Diseases, University of California, San Francisco, CA
                [2 ]Department of Neurology, University of California, San Francisco, CA
                [3 ]Colorado Division of Wildlife, Wildlife Research Center, Fort Collins, CO
                [4 ]Department of Epidemiology and Biostatistics, University of California, San Francisco, CA
                [5 ]Department of Pathology, University of California, San Francisco, CA
                Author notes
                [* ]Correspondence to Stanley B. Prusiner: Institute for Neurodegenerative Diseases, University of California, 513 Parnassus Ave, HSE-774, San Francisco, CA 94143-0518. Tel: (415) 476-4482; Fax: (415) 476-8386., stanley@ 123456ind.ucsf.edu
                Article
                nihpa133049
                10.1038/nature08289
                3186440
                19741608
                b7a223fb-930d-4303-b9ef-2431e0dae3b2
                History
                Funding
                Funded by: National Institute on Aging : NIA
                Award ID: P01 AG002132-26 || AG
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