Susceptibility of Sheep for Scrapie as Assessed by In Vitro Conversion of Nine Naturally Occurring Variants of PrP

Bossers, Alex; Vries, R. de; Smits, M.A.

doi:10.1128/jvi.74.3.1407-1414.2000

Cited by 115 publications

(120 citation statements)

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“…The first attempts at the molecular discrimination of BSE and scrapie in sheep were made in the late 1990s and early 2000s (Bosser et al, 2000;Buitkamp and Semmer, 2004). Thanks to monoclonal antibodies obtained over the years for different epitopes of the prion protein, differences were detected in the pathological material from natural cases of ovine scrapie and experimental infections of sheep with BSE (Hill et al, 1998;Kuczius et al, 1998).…”

Section: Discussionmentioning

confidence: 99%

Frequency of genotypes in the PrP prion protein gene locus in the polish sheep population

Rejduch

Knapik

Piestrzyńska-Kajtoch

et al. 2009

Acta Veterinaria Hungarica

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Scrapie is an invariably fatal transmissible neurodegenerative disease of sheep, goats and moufflons, characterised by ataxia, lower body weight and changes in behaviour. The aim of this study was to determine the genotype distribution at codons 136, 154 and 171 of the PrP locus in sheep bred in Poland. The genotypes of 801 sheep representing 10 different breeds and crossbreds were analysed using real-time PCR allele discrimination method. The combination of point mutations of the three codons (136, 154 and 171) created twelve different genotypes in the PrP locus classified into five categories of risk. The highest frequency for the ARR/ARR genotype was characteristic of the Pogórze and Berrichon du Cher breeds (72.22% and 63.25%, respectively). In the Romanov breed (1.52%) and in crossbred sheep (2.61%) the VRQ/VRQ genotype was observed.Key words: Sheep, PrP gene, molecular methods, frequency of genotypes, frequency of alleles Scrapie is an invariably fatal transmissible neurodegenerative disease of sheep, goats and moufflons, characterised by ataxia, lower body weight and changes in behaviour. Other transmissible spongiform encephalopathies (TSEs) are bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease (CWD) in deer, and Creutzfeldt-Jacob disease (including vCJD and nvCJD) in humans (Goldmann et al., 2005). The lesions appear mainly in the nervous system in the form of vacuoles triggered by the conversion of the cellular prion pro-

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Section: Discussionmentioning

confidence: 99%

Frequency of genotypes in the PrP prion protein gene locus in the polish sheep population

Rejduch

Knapik

Piestrzyńska-Kajtoch

et al. 2009

Acta Veterinaria Hungarica

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“…Therefore, further research is required to evaluate the possible consequences of selecting for resistance in large populations (Elsen et al, 1999). Bossers et al (2000) suggested that selection for several PrP variants associated with resistance would be a safer strategy and would contribute to a more genetically diverse sheep population.…”

Section: Introductionmentioning

confidence: 99%

Association between PrP genotypes and performance traits in a Welsh Mountain flock

Pritchard

Cahalan

Dewi

2008

Animal

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The UK national scrapie plan (NSP) for sheep is based on selection for the resistant ARR/ARR genotype and elimination of susceptible types of the ovine prion protein (PrP) gene. The aim of this study was to estimate the possible association of the PrP genotype and performance traits by using data from the CAMDA Welsh Mountain flock. Four alleles (ARH, ARQ, ARR and VRQ) and 10 genotypes covering all five NSP risk groups were present in the CAMDA flock. Overall, the most common allele was ARR (35.2%), and VRQ was the least common (5.4%). The commonest genotypes were ARR/ARQ (23.7%) and ARR/AHQ (23.1%). The most resistant genotype, ARR/ARR, and the most susceptible genotype, VRQ/VRQ, were found in 10.2% and 0.3%, respectively, of the population tested. The associations of PrP genotypes with weight and ultrasonically scanned traits were investigated in three analyses, the first using genotypes, the second using risk categories and the third using number of alleles. These associations were evaluated by univariate analysis of each trait using an animal model with maternal effects where appropriate, and PrP was included as a fixed effect. Selection for scrapie resistance will not adversely affect progress in the traits considered and is consistent with improvements in muscle depth.

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“…Animal transmissible spongiform encephalopathies include transmissible mink encephalopathy, scrapie of sheep and goats, chronic wasting disease of deer and elk, feline spongiform encephalopathy, and bovine spongiform encephalopathy (BSE) (1). Central to the development of these diseases is the accumulation of an infectious protease-resistant isoform (PrP Sc ) of the host-encoded cellular prion protein (PrP C ) in tissues of the central nervous system (2)(3)(4)(5).…”

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confidence: 99%

Prion protein gene (PRNP) variants and evidence for strong purifying selection in functionally important regions of bovine exon 3

Seabury

Honeycutt²,

Rooney³

et al. 2004

Proc. Natl. Acad. Sci. U.S.A.

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Amino acid replacements encoded by the prion protein gene (PRNP) have been associated with transmissible and hereditary spongiform encephalopathies in mammalian species. However, an association between bovine spongiform encephalopathy (BSE) and bovine PRNP exon 3 has not been detected. Moreover, little is currently known regarding the mechanisms of evolution influencing the bovine PRNP gene. Therefore, in this study we evaluated the patterns of nucleotide variation associated with PRNP exon 3 for 36 breeds of domestic cattle and representative samples for 10 additional species of Bovinae. The results of our study indicate that strong purifying selection has intensely constrained PRNP over the long-term evolutionary history of the subfamily Bovinae, especially in regions considered to be of functional, structural, and pathogenic importance in humans as well as other mammals. The driving force behind this intense level of purifying selection remains to be explained.T ransmissible spongiform encephalopathies, or prion diseases, are inevitably fatal neurodegenerative diseases that occur in humans as well as domestic and wild animals (1-3). Traditionally, human spongiform encephalopathies have been classified into Creutzfeldt-Jakob disease (CJD), GerstmannSträusler-Scheinker disease, and kuru, with more recent classification into variant CJD (1). Animal transmissible spongiform encephalopathies include transmissible mink encephalopathy, scrapie of sheep and goats, chronic wasting disease of deer and elk, feline spongiform encephalopathy, and bovine spongiform encephalopathy (BSE) (1). Central to the development of these diseases is the accumulation of an infectious protease-resistant isoform (PrP Sc ) of the host-encoded cellular prion protein (PrP C ) in tissues of the central nervous system (2-5).The prototypical transmissible spongiform encephalopathy, scrapie, has been observed in European sheep for Ͼ200 years, whereas BSE in domestic cattle (Bos taurus and Bos indicus; hereafter, cattle) dates to 1986, presumably resulting from scrapie-and͞or BSE-infected cattle feed (6-9). Thus, BSE seems to be a more recent phenomenon associated with modern agricultural practices. This idea is supported by several lines of evidence. First, at least 14 amino acid polymorphisms encoded by exon 3 of the ovine prion protein gene (PRNP) have been described (10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20), and those associated with codons 136 and 171 have been shown to influence expression of scrapie (8, 10). However, no amino acid polymorphisms associated with BSE have been identified in cattle, although an insertion-deletion (indel) polymorphism in the putative bovine PRNP promoter was shown to exhibit an association with BSE in a few German cattle breeds (21). Second, recorded patterns of variation within cattle PRNP exon 3 are markedly different from those observed in sheep, with variability in cattle primarily restricted to 11 synonymous nucleotide sites and two nonsynonymous sites where low-frequency variation has been observed (2...

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Susceptibility of Sheep for Scrapie as Assessed by In Vitro Conversion of Nine Naturally Occurring Variants of PrP

Cited by 115 publications

References 36 publications

Frequency of genotypes in the PrP prion protein gene locus in the polish sheep population

Frequency of genotypes in the PrP prion protein gene locus in the polish sheep population

Association between PrP genotypes and performance traits in a Welsh Mountain flock

Prion protein gene (PRNP) variants and evidence for strong purifying selection in functionally important regions of bovine exon 3

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