Gene/Protein Disease Symptom Drug Enzyme Compound
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In February 2003, a postal survey of 1279 sheep farmers in the Shetland Islands yielded 586 responses (46 per cent response rate). The principal aim of the survey was to gather information on the history and control of scrapie. Overall, 28.5 per cent of the respondents thought they had had a case of scrapie in their flock at some time. There was a slow increase in the proportion of affected flocks during the 1970s, followed by a more rapid increase during the 1980s and early 1990s, and a decline from the mid-1990s onwards. The peak proportion of affected flocks was approximately 6 per cent in 1994. Of the farmers who had ever had scrapie in their flock, 97.1 per cent had attempted to control the disease. The most common method of control was breeding from non-susceptible tups, used by 90.6 per cent of the affected flocks and 75.1 per cent of the flocks that had never been affected. A comparison of the characteristics of the affected and unaffected flocks indicated that an increased risk of scrapie was associated with the larger flocks, the open flocks and the flocks that bought in lambs. The basic reproduction ratio for the spread of scrapie between flocks was estimated to be 1.47, and the mean duration of an outbreak within a flock was estimated to be approximately two years.
Vet Rec 2006 Feb 25
PMID:Results of a postal survey of scrapie in the Shetland Islands in 2003. 1650 Nov 56

Samples of tissue from the central nervous system (cns), the lymphoreticular system (lrs) and the rectal mucosa of a large number of scrapie-exposed sheep, with and without signs of clinical disease, were examined immunohistochemically for evidence of disease-associated prion protein (PrP(d)). The rectal mucosa has received almost no attention so far in scrapie diagnosis, despite its abundant rectoanal mucosa-associated lymphoid tissue, and its accessibility. The scrapie-confirmed cases included 244 with clinical disease, of which 237 (97.1 per cent) were positive in the rectal mucosa, and 121 apparently healthy sheep, of which 104 (86 per cent) were positive in the rectal mucosa. PrP(d) was detected in 86.4 to 91.5 per cent of the other lrs tissues of the healthy sheep examined and in 77.7 per cent of their cns tissues. The stage of infection, therefore, affected the probability of a positive result in the rectal mucosa, whereas the breed, PrP genotype, age and sex had little or no independent effect. Accumulations of PrP(d) were observed in the rectal mucosa and other lrs tissues of vrq/arr sheep with preclinical and clinical scrapie, albeit with a lower frequency and magnitude than in sheep of other PrP genotypes. Western immunoblotting analyses of samples of rectal mucosa gave the characteristic PrP glycoprofile, with a sensitivity similar to that of immunohistochemistry.
Vet Rec 2006 Mar 11
PMID:Postmortem diagnosis of preclinical and clinical scrapie in sheep by the detection of disease-associated PrP in their rectal mucosa. 1653 80

Dicalcium phosphate was prepared from industrial crushed bone artificially contaminated with transmissible spongiform encephalopathy agents in two experiments carried out in an accurately scaled-down laboratory model of the industrial manufacturing process. In one experiment, 10 g of mouse brain infected with the 301V strain of mouse-passaged bovine spongiform encephalopathy agent was added to the crushed bone; in the other experiment, 10 g of hamster brain infected with the 263K strain of hamster-passaged scrapie agent was added. Samples of the infectious brain and dried dicalcium phosphate were assayed for the amount of 301V or 263K infectivity present. The titre of infectivity of the 301V-infected brain was 10(7.7) intracerebral ID50/g; that of the 263K-infected brain was 10(8.0) intracerebral ID50/g. The titres of the dried samples of dicalcium phosphate were 10(2.5) ID50/g in the experiment spiked with 301V and 10(2.7) ID50/g in the experiment spiked with 263K. The calculated clearance factors were 10(3.9) for the experiment with 301V and 10(3.8) for the experiment with 263K.
Vet Rec 2006 Mar 18
PMID:Inactivation of transmissible spongiform encephalopathy agents during the manufacture of dicalcium phosphate from bone. 1654 82

An anonymous postal survey was conducted in 2002 to estimate the proportion of farms in Great Britain affected with scrapie and to gather information on the likely risk factors for the occurrence of the disease; the response rate was 53 per cent. The survey showed that 1 per cent of the respondents thought they had had scrapie in their flock in the previous 12 months, and that 12 per cent thought they had had scrapie in the past. The results of the survey were consistent with the results of a similar survey carried out in 1998, and with notification patterns, but in 1998 approximately 3 per cent of farmers reported having had scrapie in the previous 12 months. It is not clear whether the apparent decrease in the prevalence of scrapie is real or whether it may be due to factors such as sampling biases, or to the increasing knowledge of the signs of scrapie shown by the respondents in 2002.
Vet Rec 2006 Apr 15
PMID:Descriptive analysis of the results of an anonymous postal survey of the occurrence of scrapie in Great Britain in 2002. 1661 40

A total of 214 sheep with lesions of the oral mucosa were recorded at the Veterinary Laboratories Agency regional laboratories between March 15, 2002 and February 28, 2003. Using denominator data from the scrapie abattoir survey a prevalence of 0.95 per cent (95 per cent CI 0.82 to 1.10 per cent) was recorded, and using the fallen stock survey a prevalence of 1.15 per cent (95 per cent CI 0.64 to 1.91 per cent) was recorded. The lesions varied widely and included dental pad lesions in 20 per cent of cases. In total, 248 sites were affected, the commonest being the lower gum below the incisors, which was affected in 100 sheep. Most of the lesions were 1 cm or less in diameter. Ninety per cent of the 251 lesions examined histologically were erosions, ulcers, healed ulcers, focal epithelial necrosis or haemorrhages, and the changes observed indicated that trauma was the most likely primary cause.
Vet Rec 2006 May 20
PMID:Characteristics, prevalence and aetiology of lesions of the oral mucosa in adult sheep. 1671 32

The Cheviot flock at the Institute for Animal Health's Neuropathogenesis Unit (npu) has endemic scrapie, which affects primarily vrq/vrq sheep and at high frequency. A new flock with a full range of PrP genotypes, including the highly susceptible vrq/vrq, has been produced on a separate site, from animals in the npu breeding flock, and it remains scrapie-free after eight years. In contrast, in a parallel flock at the npu farm, scrapie has reappeared after five years, although the animals were kept in separate accommodation from the scrapie-affected sheep. During this time the npu breeding flock continued to have scrapie cases. Although it is known that highly susceptible sheep can remain free of infection in a clean environment, this is the first report of the infection being removed successfully from the bloodlines of scrapie-affected sheep. The results confirm that scrapie is not a genetic disease dependent only on the PrP gene sequence, but requires both genetic susceptibility and an infectious agent.
Vet Rec 2006 Jul 08
PMID:Derivation of a scrapie-free sheep flock from the progeny of a flock affected by scrapie. 1682 98

The spatial distribution of sheep flocks in Great Britain with confirmed clinical scrapie between January 1993 and December 2002 inclusive was investigated by using kernel density estimation and a cluster scan test statistic. Six statistically significant clusters were identified: three were lower risk, and were centred on the north-western coast of Scotland, the north-western coast of Wales and the South Yorkshire/Pennine region; three were of higher risk, and were centred in the central south, North Yorkshire and north Cumbria. General knowledge and the results of previous epidemiological studies were used to generate biologically plausible hypotheses that might explain these findings. They included aspects of flock management and disease transmission, and factors associated with the identification of cases, including their detection, recognition and, in particular, reporting levels, as well as diagnosis and animal movements.
Vet Rec 2006 Aug 05
PMID:Descriptive spatial analysis of scrapie-affected flocks in Great Britain between January 1993 and December 2002. 1701 14

A total of 167 sheep belonging to the Estonian whiteheaded mutton, Estonian blackheaded mutton, Lithuanian coarsewool native, Lithuanian blackface and Latvian darkheaded mutton breeds, and a population of sheep kept isolated on the Estonian island of Ruhnu, were sequence-analysed for polymorphisms in the prion protein (PrP) gene, to determine their genotype and the allele frequencies of polymorphisms in PrP known to confer resistance to scrapie. A 939 base pair fragment of exon 3 from the PrP gene was amplified by pcr and analysed by direct sequencing. For animals showing polymorphism at two nucleotide positions, both haplotypes of these double-heterozygous genotypes were further verified by pcr cloning and sequence analysis. Known polymorphisms were observed at codons 136, 154 and 171, and six different haplotypes (arr, ahq, arh, ahr, arq and vrq) were determined. On the basis of these polymorphisms, the six populations of sheep possessed the resistant arr haplotype at different frequencies. The high-risk arq haplotype occurred in high frequencies in all six populations, but vrq, the haplotype carrying the highest risk, occurred at low frequencies and in only three of the populations.
Vet Rec 2006 Aug 19
PMID:Detection of prion protein gene polymorphisms in Baltic breeds of sheep. 1692 Oct 14

The National Scrapie Plan (NSP) for Great Britain is a voluntary scheme that, through PrP genotype testing and restricted breeding, aims to reduce the risk of scrapie in the national sheep flock. To gauge the progress in member flocks and within breeds, the genotype profile of successive crops of ram lambs was monitored between 2002 and 2004. In each of the 11 most frequently sampled breeds, the proportion of ram lambs testing in the most resistant genotype category (NSP type 1: ARR/ARR) increased, and there was a reduction in the proportion of genotypes associated with the highest disease risk, that is X/VRQ, where X is an allele other than ARR. Changes in the proportion of ram lambs testing for ARR-heterozygous genotypes (NSP type 2: ARR/X, where X is not VRQ) appeared to be influenced by whether the sheep were hill breeds or non-hill breeds. In each of six frequently sampled hill breeds these genotypes expanded, in relative terms, whereas they declined in four of five prominent lowland/crossing breeds. The proportion of ram lambs that carried neither ARR nor VRQ (NSP type 3) declined consistently in the top 11 breeds, but there was little change in the ARR/VRQ genotype (NSP type 4). Among individual flocks that had ram lambs tested in all three years 2002 to 2004, the majority experienced an increase in the proportion testing ARR/ARR, and of those that had VRQ ram lambs in 2002, most recorded a decrease in their frequency by 2004.
Vet Rec 2006 Oct 07
PMID:PrP genotype progression in flocks participating in the National Scrapie Plan for Great Britain. 1702 48

Reports of clinical scrapie in Great Britain between January 1, 1993 and December 31, 2002 were reviewed. Scrapie was confirmed in 4142 sheep on 1099 holdings. The cumulative case and holding incidence risks decreased in 2001, probably owing to the outbreak of foot-and-mouth disease, although there were regional variations. Sheep aged between three and four years old constituted the largest affected group. In the period between 1998 and 2002, 51.3 per cent of the cases had the genotype ARQ/VRQ, 19.3 per cent were ARQ/ARQ and 18.9 per cent were VRQ/VRQ; Swaledale, Shetland and Welsh mountain sheep were the most common pure breeds reported. The areas at highest risk were the Shetland Islands, followed by the south and east of England.
Vet Rec 2006 Dec 09
PMID:Analysis of data from the passive surveillance of scrapie in Great Britain between 1993 and 2002. 1715 10


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