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Because bovine spongiform encephalopathy (BSE) has probably been caused by accidental transmission of the transmissible agent of sheep scrapie there is concern that humans may be at risk from BSE. Epidemiological and experimental evidence is examined which suggests that this is unlikely.
Vet Rec 1989 Oct 14
PMID:Bovine spongiform encephalopathy and human health. 268 50

An isolate of scrapie designated CH1641 was identified from a natural case of scrapie in a Cheviot sheep by passage in sheep and goats. It has not been possible to transmit scrapie to mice from this source. The Sip gene which controls the incubation periods of experimental scrapie in Cheviot sheep has two alleles; sA which shortens and pA which lengthens the incubation periods of most strains of scrapie after the first experimental injection in sheep (the A group of strains). The CH1641 isolate differs from them in that the alleles of Sip act in the opposite way, with incubation being shorter in the pA homozygotes. There is some evidence that one or more genes, in addition to Sip, may be implicated in the control of scrapie incubation in sheep and the possibility of a carrier infection with CH1641 is also discussed.
Vet Rec 1988 Jul 02
PMID:The unusual properties of CH1641, a sheep-passaged isolate of scrapie. 314 Apr 68

This study, initiated in June 1987, describes the epidemiology of bovine spongiform encephalopathy (BSE), a recently described novel neurological disease of domestic cattle first identified in Great Britain in November 1986. Records suggested that the earliest suspected cases occurred in April 1985. There was variability in the presenting signs and the disease course, but the majority of cases developed behavioural disorders, gait ataxia, paresis and loss of bodyweight; pruritus was not a predominant sign. The form of the epidemic was typical of an extended common source in which all affected animals were index cases. The use of therapeutic or agricultural chemicals on affected farms presented no common factors. Specific genetic analyses eliminated BSE from being exclusively determined by simple mendelian inheritance. Neither was there any evidence that it was introduced into Great Britain by imported cattle or semen. The study supports previous evidence of aetiological similarities between BSE and scrapie of sheep. The findings were consistent with exposure of cattle to a scrapie-like agent, via cattle feedstuffs containing ruminant-derived protein. It is suggested that exposure began in 1981/82 and that the majority of affected animals became infected in calfhood.
Vet Rec 1988 Dec 17
PMID:Bovine spongiform encephalopathy: epidemiological studies. 321 47

Previous research has consistently demonstrated by electron microscopy the presence of scrapie associated fibrils in brain extracts prepared from mice and hamsters with clinical signs of experimental scrapie. In the present study similar fibrils were seen in all the brain extracts prepared from 11 Cheviot or Suffolk sheep with natural or experimental scrapie that had been diagnosed clinically and confirmed neuropathologically. They were not found in the brain extracts of nine sheep that did not have scrapie and which included four that had been injected with infected material but did not develop the disease. The presence of such fibrils can therefore be used as an additional diagnostic criterion for natural scrapie in sheep.
Vet Rec 1987 Feb 07
PMID:Scrapie associated fibrils in the diagnosis of scrapie in sheep. 360 27

A flock of 294 Swaledale sheep was injected with brain tissue from different sources so as to include strains of natural scrapie currently affecting the Swaledale breed. Discounting intercurrent deaths, 85 per cent of the flock developed clinical scrapie within an observation period of 2557 days (seven years). Scrapie cases fell into distinct early and late groups, occurring at (mean +/- sem) 287 +/- 7 days and 1207 +/- 38 days after injection, respectively. Breeding groups were formed from the survivors after the early cases had occurred and successive lamb crops were produced. Two groups of progeny and two groups of controls were injected with scrapie. The incidence of scrapie in the progeny (27 per cent) was much lower than in the controls (64 per cent). The incidence in the progeny was also assessed in relation to the response of their parents: it was high (about 40 per cent) when one or both parents developed late scrapie but otherwise very low (about 5 per cent). Hence the progeny of surviving parents had a greatly reduced susceptibility to the strains of scrapie used. Seventy-two of these selected progeny formed a nucleus flock which is being bred at Redesdale. The next stage of the experiment is to assess the effect of selected rams in recorded flocks with a high incidence of natural scrapie.
Vet Rec 1985 Feb 23
PMID:Selection of Swaledale sheep of reduced susceptibility to experimental scrapie. 398 98

Since 1986, scrapie-like spongiform encephalopathy has been diagnosed in 19 captive wild animals of eight species at or from eight zoological collections in the British Isles. The affected animals have comprised members of the family Bovidae: one nyala (Tragelaphus angasi), four eland (Taurotragus oryx), and six greater kudu (Tragelaphus strepsiceros), one gemsbok (Oryx gazella), one Arabian oryx (Oryx leucoryx), and one scimitar-horned oryx (Oryx dammah), and members of the family Felidae: four cheetah (Acinonyx jubatus) and one puma (Felis concolor). In addition, three cases of a spongiform encephalopathy of unknown aetiology have been reported in ostriches (Struthio camellus) from two zoos in north west Germany. Three features suggest that some of these cases may have been caused by the agent of bovine spongiform encephalopathy (BSE). First, they have been temporally and geographically coincident with the BSE epidemic. Secondly, in all the ungulates for which details are available, it is possible that either the affected animal itself, or the herd into which it was born or moved, had been exposed to proprietary feeds containing ruminant-derived protein or other potentially contaminated material, and all the carnivores had been fed parts of cattle carcases judged unfit for human consumption. Thirdly, the pathological results of inoculating mice with a homogenate of fixed brain tissue from the nyala and from one greater kudu were similar to the results of inoculating mice with BSE brain tissue.
Vet Rec 1994 Sep 24
PMID:Epidemiological observations on spongiform encephalopathies in captive wild animals in the British Isles. 784 43

Bovine spongiform encephalopathy (BSE) is one of a family of scrapie-like diseases which affect various mammals. Polymorphisms and mutations of the PrP gene have been associated with the incidence of experimental and natural scrapie in other animals and this study of the bovine PrP gene was undertaken to discover whether there was a similar association with PrP genotype in cattle with BSE. There are two known polymorphisms of the coding region of the bovine PrP gene, a silent HindII restriction site polymorphism and a difference in the number of an octapeptide repeated sequence (either five or six copies). An analysis of 370 cattle in Scotland revealed no difference between the frequencies of these PrP genotypes in healthy cattle and cattle with BSE.
Vet Rec 1994 Oct 22
PMID:Frequencies of PrP gene variants in healthy cattle and cattle with BSE in Scotland. 785 30

Spongiform encephalopathy has been confirmed in both 'positive' and 'negative' lines of Cheviot sheep (selected for their differential response on experimental exposure to scrapie) after intracerebral injection or oral dosing with brain homogenate derived from cattle with bovine spongiform encephalopathy (BSE). With either challenge the incubation period of the disease ranged from 440 to 994 days in both lines of sheep. In a similar experiment, three Anglo-Nubian goats developed the disease 506 to 570 days after intracerebral infection with the same BSE homogenate, and two of three goats developed the disease 941 and 1501 days after oral dosing; the other goat and some sheep from each of the experimental groups remain alive 1720 days after exposure. This is the first report of the experimental transmission of BSE to sheep and goats.
Vet Rec 1993 Oct 02
PMID:Transmission of bovine spongiform encephalopathy to sheep and goats. 823 76

Two independent nationwide surveys of randomly selected sheep farmers were conducted: in one a questionnaire was mailed to farmers and in the other the farmers were interviewed. The questionnaire was designed to obtain data on the occurrence of scrapie in the national sheep flock of the Netherlands. Farmers owning 30 or more ewes were selected from a database at the Ministry of Agriculture, Nature Management and Fisheries, resulting in 10,000 addresses. Two lists of names were compiled at random. In the postal survey 700 questionnaires were distributed, and 137 farmers were interviewed directly. The response rate in the postal survey was 37 per cent and in the interview survey 100 per cent. The results from the two surveys did not differ significantly. Six per cent of the farmers indicated that they had at some time observed signs of scrapie in their flock. For the total sheep population in the Netherlands, the prevalence of flocks with sheep showing signs of scrapie was between 3.8 per cent and 8.4 per cent (with 95 per cent certainty). In 1989 and 1990 the incidence rate within infected flocks was 1.27 cases/100 ewes/year, and the incidence rate for the total sample population was 0.1 case/100 ewes/year.
Vet Rec 1993 Aug 28
PMID:Prevalence and incidence of scrapie in The Netherlands: a questionnaire survey. 823 24

Two young male common marmosets (Callithrix jacchus) were injected intracerebrally and intraperitoneally with a crude brain homogenate prepared from a cow with bovine spongiform encephalopathy (BSE). Two other marmosets were similarly injected with brain homogenate from a sheep with natural scrapie. The two animals injected with scrapie material developed neurological signs 38 and 42 months after injection and the two animals injected with BSE material developed neurological signs after 46 and 47 months. Post mortem examination of the brains revealed spongiform encephalopathy especially in the basal nuclei and diencephalon of all the animals and, in addition, involvement of the cerebral cortex of the marmosets injected with scrapie material. The experiment extends the host range of experimental BSE to include a primate species.
Vet Rec 1993 Apr 17
PMID:Experimental transmission of BSE and scrapie to the common marmoset. 848 58


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