Journal of General Virology · 2004 · 85 citations · 37 references
Veterinary MedicineAnimal SciencesInfectious DiseasesMolecular EpidemiologyPrion DiseasesSheep MedicineAnimal Prion DiseasesPrion Disease DiagnosisPrion Protein MisfoldingSheep Infectious DiseasesCase-control Study DesignGel ElectrophoresisGenetic Prion Protein MutationsHistopathological LesionsPrion Protein Gene SequencingPrion Protein Gene TestingProteinase-resistant Prion ProteinSheep Diseases
A total of 216 local crossbred sheep from 16 scrapie-affected Greek flocks and 210 purebred sheep of the milk breeds Chios and Karagouniko from healthy flocks were analysed for scrapie-linked polymorphisms in the prion protein (PrP) gene. Of the 216 sheep in this case-control study, 96 sheep were clinical cases, 25 subclinical cases (asymptomatic at the moment of euthanasia but positive by histopathology and/or ELISA detecting proteinase-resistant PrP) and 95 healthy controls (negative by all evaluations). Polymorphisms at codons 136, 154 and 171 were determined by denaturing gradient gel electrophoresis, followed by RFLP and sequencing. Scrapie, both clinical and subclinical, was associated with the genotypes ARQ/ARQ (88 of 110 sheep of that genotype), ARQ/TRQ (9 of 13), ARQ/AHQ (15 of 38) and VRQ/VRQ (9 of 17). Histopathological lesions were more severe in the clinical cases. Genotypes ARQ/ARR (26 sheep), ARQ/ARK (seven sheep), AHQ/ARR (one sheep), ARH/ARH (one sheep) and ARR/ARH (three sheep) were detected exclusively in healthy control sheep. In the purebred survey, four genotypes were present in the Chios sheep (ARQ/ARQ, ARQ/TRQ, ARQ/AHQ and ARQ/ARR) and four in the Karagouniko sheep (ARQ/ARQ, ARQ/AHQ, ARQ/ARR and ARQ/ARH).
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Mice devoid of PrP are resistant to scrapie
Hansruedi Büeler, Adriano Aguzzi, Andreas W. Sailer et al. · Cell · 1993 · 2.1K citations · Full text
Nearly ubiquitous tissue distribution of the scrapie agent precursor protein
Paul E. Bendheim, H. R. Brown, R. D. Rudelli et al. · Neurology · 1992 · 468 citations
Medicine, Neurobiology, Neuroscience +15