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Transmissible Spongiform Encephalopathies (TSEs) are neurological diseases that have been found in humans, cattle, sheep and other mammals (e.g. cats, goats, deer, antelope). The agent of disease is believed to be an abnormal prion protein (or is prion associated). An abnormal prion is a protein with an abnormal folding structure when compared to the naturally occurring prion found in nervous and lymphoreticular tissues. Various abbreviations are in use to refer to this: Prion Protein (PrP); Abnormal Prion Protein (PrP<sup>ab</sup>); Resistant Prion Protein (PrP<sup>res</sup>); Scrapie Prion Protein (PrP<sup>sc</sup>) and others. In most instances the abnormal prion is resistant to protein kinase digestion, a feature used in diagnostic techniques. Clinical signs include progressive ataxia and pruritis. Histology (immunohistochemistry) usually shows vacuolation and an accumulation of prion proteins in various parts of the CNS (especially the brain and spinal cord).
Transmissible Spongiform Encephalopathies (TSEs) are neurological diseases that have been found in humans, cattle, sheep and other mammals (e.g. cats, goats, deer, antelope). The agent of disease is believed to be an abnormal prion protein (or is prion associated). An abnormal prion is a protein with an abnormal folding structure when compared to the naturally occurring prion found in nervous and lymphoreticular tissues. Various abbreviations are in use to refer to this: Prion Protein (PrP); Abnormal Prion Protein (PrP<sup>ab</sup>); Resistant Prion Protein (PrP<sup>res</sup>); Scrapie Prion Protein (PrP<sup>sc</sup>) and others. In most instances the abnormal prion is resistant to protein kinase digestion, a feature used in diagnostic techniques. Clinical signs include progressive ataxia and pruritis. Histology (immunohistochemistry) usually shows vacuolation and an accumulation of prion proteins in various parts of the CNS (especially the brain and spinal cord).
BSE - a known human health risk - was confirmed in one goat slaughtered in France in 2002; and probably present in one UK goat born in 1987. Another UK goat killed in 2008 remains under investigation as BSE could not be excluded on standard tests. Naturally occurring BSE has not been recorded in sheep, and based on the results of extensive active surveillance (currently 10,000 fallen sheep, 500 fallen goats and 10,000 sheep intended for human consumption per annum). The UK’s independent Spongiform Encephalopathy Advisory Committee (SEAC) concluded in 2007, that the UK prevalence was probably 0, or at the worst 10 flocks might be affected.
BSE - a known human health risk - was confirmed in one goat slaughtered in France in 2002; and probably present in one UK goat born in 1987. Another UK goat killed in 2008 remains under investigation as BSE could not be excluded on standard tests. Naturally occurring BSE has not been recorded in sheep, and based on the results of extensive active surveillance (currently 10,000 fallen sheep, 500 fallen goats and 10,000 sheep intended for human consumption per annum). The UK’s independent Spongiform Encephalopathy Advisory Committee (SEAC) concluded in 2007, that the UK prevalence was probably 0, or at the worst 10 flocks might be affected.
[[Category:Transmissible Spongiform Encephalopathies]]