
Scrapie
Scrapie is a deadly, degenerative and transmissible disease that affects the central nervous system of sheep and goats.
Scrapie has been known since the 18th century. This disease has been present in sheep and goats for centuries and has been observed worldwide, but especially in Western Europe and North America. In French the disease is called Tremblante and in German Traberkrankheit.
Severe itching
The name Scrapie is derived from one of the symptoms. Due to severe itching, sick animals scrape their skin against fences or other fixed objects. Other symptoms are a dry coat and skin, and severe weight loss. Movement disorders can also occur. However, these are not as pronounced as with BSE in cattle; usually only one animal of the herd is affected.
Long incubation time
Scrapie has an incubation period of more than two years. As a result, the disease symptoms are only seen in adult animals. In non-susceptible animals, the pathogen has virtually no chance of causing disease symptoms during their relatively short lives.
Prion disease
There are classical forms and atypical forms of scrapie. Scrapie, like BSE, is a transmissible spongiform encephalopathy (TSE) or prion disease. Prion diseases are very unusual; unlike bacterial, viral or parasitic infections, prion diseases are caused by a protein that is normally present in the host. This protein is known as a prion protein or simply PrP.
Spreading the infection
Scrapie is an infectious disease. The nature of the agent (the prion) that causes Scrapie is not fully understood, and the mode of transmission is not entirely clear. It has been established that the Scrapie agent can survive several years in the environment, that it is relatively resistant to disinfectants and that host genetic (hereditary) factors mainly determine the susceptibility to the disease. In sheep, the assumption is that an important route of horizontal and vertical transmission is via the placental material (afterbirth) of infected ewes during lambing.
Publications
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Scrapie prevalence in sheep of susceptible genotype is declining in a population subject to breeding for resistance
BMC Veterinary Research 6 (2010). - ISSN 1746-6148 -
Human Variant Creutzfeldt-Jakob disease and sheep scrapie PrP (res) detection using seeded conversion of recombinant prion protein.
Protein Engineering, Design & Selection 22 (2009)8. - ISSN 1741-0126 - p. 515 - 521. -
State-of-the-art review of goat TSE in the European Union, with special emphasis on PRNP genetics and epidemiology
Veterinary Research 40 (2009)5. - ISSN 0928-4249 - p. 48 - 48. -
Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease: its effect on the phenotype and prion-type characteristics
Brain 132 (2009). - ISSN 0006-8950 - p. 2643 - 2658. -
Prion protein self-peptides modulate prion interactions and conversion
BMC Biochemistry 10 (2009). - ISSN 1471-2091 - 16 p. -
Atypical scrapie in Italy: immunohistochemical study with a panel of different antibodies
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TSE pathogenesis in cattle and sheep
Veterinary Research 39 (2008)4. - ISSN 0928-4249 - 12 p. -
Pathogenesis of bovine spongiform encephalopathy in sheep
Archives of Virology 153 (2008)3. - ISSN 0304-8608 - p. 445 - 453. -
Genetic variability of the PRNP gene in goat breeds from Northern and Southern Italy
Journal of Applied Microbiology 104 (2008)6. - ISSN 1364-5072 - p. 1782 - 1789. -
Identification of early natural scrapie-specific gene expression changes in tonsil and peyers patches of sheep using a sheep DNA microarray