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Scrapie usually affects sheep around three to five years of age.<ref>{{Cite web |last=Animal and Plant Health Inspection Service |date=June 2, 2020 |title=Scrapie Disease Information |url=https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/sa_animal_disease_information/sheep-goat/disease-info/index |website=U.S. DEPARTMENT OF AGRICULTURE}}</ref> The potential for transmission at birth and from contact with placental tissues is apparent.<ref>{{
==Regulation==
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The disease has been notifiable in the EU since 1993, but unlike [[Bovine spongiform encephalopathy|BSE]], there was no evidence as of 1999 to suggest that scrapie is a risk to human health.<ref name="Brown et al., 1987">Brown P, Cathala F, Raubertas RF, Gajdusek DC, Castaigne P (1987) "The epidemiology of Creutzfeldt–Jakob disease: conclusion of a 15-year investigation in France and review of the world literature". ''Neurology'', 37(6):895–904.</ref><ref name="Harries et al.,1988">Harries JR, Knight R, Will RG, Cousens SN, Smith PG, Mathews WB (1988) "Creutzfeldt–Jakob disease in England and Wales, 1980–1984: a case-control study of potential risk factors". ''Journal of Neurology Neurosurgery and Psychiatry'', 51(9):1113–1119.</ref><ref name="Kondo and Kuriowa, 1982">Kondo K, Kuriowa Y (1982). "A case control study of Creutzfeldt-Jakob disease: association with physical injuries". ''Annals of Neurology'', 11(4):377–381.</ref><ref name="WHO, 1999">World Health Organization, 1999. WHO consultation on public health and animal transmissible spongiform encephalopathies: epidemiology, risk and research requirements, with the participation of the Office International des Epizooties. [http://www.who.int/csr/resources/publications/bse/WHO_CDS_CSR_APH_2000]{{dead link|date=December 2021|bot=medic}}{{cbignore|bot=medic}} Accessed 7 March 2005. http://www.who.int/csr/resources/publications/bse/en/whocdscsraph20002.pdf.</ref> In July 2003, a [[Canadian Food Inspection Agency]] officer said that while scrapie shows up every year on Canadian farms, "We've had a lot of experience with scrapie and there's never been a link between scrapie and human illness."<ref name=npnsp/> As of 2004, the [[USDA]] made no mention of scrapie in its ''Sheep and Goats Death Loss'' circular.<ref name="nass">{{cite web| date= 2005-05-06 |title=Sheep and Goats Death Loss | publisher=[[National Agricultural Statistics Service]] |url=https://downloads.usda.library.cornell.edu/usda-esmis/files/hh63sv88v/xk81jp327/9g54xm03j/sgdl-05-06-2005.pdf |access-date=2019-01-22}}</ref>
Historically, scrapie had been considered to be an animal health issue. However, between 1996 and 1999, the UK Spongiform Encephalopathy Advisory Committee had been considering the control and eradication of scrapie in the UK also with public health in mind because of concern over five issues:<ref name="defra">{{cite web|url=http://www.defra.gov.uk/animalh/bse/othertses/scrapie/nsp/pdf/benefitsrealisation021206.pdf|archive-url=http://webarchive.nationalarchives.gov.uk/20081109071125/http://www.defra.gov.uk/animalh/bse/othertses/scrapie/nsp/pdf/benefitsrealisation021206.pdf|url-status=dead|archive-date=2008-11-09|title=National Scrapie Plan / Ram Genotyping Scheme Review / Benefits Review|publisher=[[Department for the Environment, Food and Rural Affairs]]|date=December 2006|access-date=2019-01-22}} [[File:UKOpenGovernmentLicence.svg|30px]] This article contains quotations from this source, which is available under the [http://webarchive.nationalarchives.gov.uk/20130402161723/http://www.nationalarchives.gov.uk/doc/open-government-licence/ Open Government Licence v1.0]. © Crown copyright.</ref>
* [[Meat and bone meal]] (MBM), which was suspected to be the source of the BSE in cattle outbreak in the late 1990s, had also been fed to sheep and goats.
* BSE had been transmitted to sheep following experimental oral exposure.
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==Clinical signs and diagnosis==
Changes are mild at first; slight behavioral changes and an increase in chewing movements may occur. Ataxia and neurological signs then develop, and affected sheep struggle to keep up with the flock.<ref name="WikiVet">
The signs and effects of scrapie typically appear 2-5 years after infection but may appear afterwards. Once the onset of clinical signs has occurred, sheep typically live for 1-6 months. In some cases, they may live longer, but death is an inevitable consequence of the condition. Signs of scrapie vary between infected individual animals and develop slowly. Due to the nerve cell damage caused by the condition, affected animals may exhibit behavioral changes, tremor, [[pruritus]], and locomotor incoordination.<ref>{{cite web |title=Scrapie |url=https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/nvap/NVAP-Reference-Guide/Control-and-Eradication/Scrapie |website=Animal and Plant Health Inspection Service |access-date=27 February 2023}}</ref>
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In the [[UK]], the [[Second Blair ministry|Blair ministry]] implemented in 2001 a ''National Scrapie Plan'', which encouraged breeding from sheep that are genetically more resistant to scrapie. This is intended to eventually reduce the incidence of the disease in the UK sheep population.<ref>{{cite journal|pmid=11688751|year=2001|last1=Atkinson|first1=M.|title=National scrapie plan|journal=The Veterinary Record|volume=149|issue=15|pages=462}}</ref><ref>{{cite web |url=http://ec.europa.eu/competition/state_aid/cases/138139/138139_1153266_4_2.pdf|title=Letter from Franz FISCHLER, European Commission Directorate-General for Agriculture to The Right Hon Jack Straw MP Secretary of State for Foreign and Commonwealth Affairs regarding State aid N 544/2002 National Scrapie Plan for Great Britain: Phase 1 – Genotyping schemes to encourage breeding for genetic resistance}}</ref> Scrapie occurs in [[Europe]] and [[North America]], but to date, [[Australia]] and [[New Zealand]] (both major sheep-producing countries) are scrapie-free.<ref>{{
Breeds such as [[Cheviot sheep|Cheviot]] and [[suffolk (sheep)|Suffolk]] are more susceptible to scrapie than other breeds.<ref name=Straiton>Eddie Straiton, "Sheep Ailments - recognition and treatment", 7th edition (2001) {{ISBN|1-86126-397-X}}</ref> Specifically, this is determined by the genes coding for the naturally occurring prion proteins. The most resistant sheep have a double set of ''ARR'' [[allele]]s, while sheep with the ''VRQ'' allele are the most susceptible.<ref name=Helse>Synnøve Vatn, Lisbeth Hektoen, Ola Nafstad "Helse og Velferd hos sau" 1. utgave, Tun Forlag (2008) {{ISBN|978-82-529-3180-8}}</ref> A simple blood test reveals the allele of the sheep, and many countries are actively breeding away the ''VRQ'' allele.
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===Exposure through contaminated vaccines===
* '''Contaminated Louping-ill vaccine
* '''Contaminated Mycoplasma agalactiae vaccine'''.
===Exposure through contaminated soil===
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===Transmission summary===
Prions (PrP<sup>Sc</sup>) are shed from sheep and goats in birth fluids, feces and other excrement. The concentration of the prions is uncertain, but is not directly proportional to infectivity. Sheep ingest a considerable amount of soil, so soil represents a plausible environmental reservoir of scrapie prions, which can persist in the environment for years. Longevity of the prions and the attachment of soil particles likely influences the persistence and infectivity of prions in the environment.<ref>{{
Effective methods of inactivating prions in the soil are currently lacking, and the effects of natural degradation mechanisms on prion infectivity are largely unknown. An improved understanding of the processes affecting the mobility, persistence and [[bioavailability]] of prions in soil is needed for the management of prion-contaminated environments. A system for estimating the prion-binding capacity of soil on farms using simple soil analysis may allow an estimate of the prion risk in the environment, and whether altering prion binding by the use of soil amendments may help to mitigate the infectious prions. [[Lichen]]s, specifically, ''[[Parmelia sulcata]]'', ''[[Cladonia rangiferina]]'' and ''[[Lobaria pulmonaria]]'', may have potential for reducing the number of prions because some lichen species contain [[protease]]s that show promise in breaking down the prion. Further work to clone and characterize the proteases, assess their effects on prion infectivity, and determine which component organism or organisms present in lichens produce or influence the protease activity is warranted and is currently under investigation.<ref>{{cite journal|last=Johnson|first=CJ|title=Degradation of the disease-associated prion protein by a serine protease from lichens|journal=PLOS ONE|year=2011|doi=10.1371/journal.pone.0019836|volume=6|issue=5|pages=e19836|display-authors=etal|pmid=21589935|pmc=3092769|bibcode=2011PLoSO...619836J|doi-access=free}}</ref>
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* [[Transmissible mink encephalopathy]]
* [[Virino]]
* [[Rams (2015 film)|''Rams'' (film)]], a
== References ==
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