| Determination of the frequency and distribution of vascular and parenchymal amyloid with polyclonal and N-terminal-specific PrP antibodies in scrapie-affected sheep and mice. | |
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MedLine Citation:
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PMID: 9637378 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Brains from 17 histopathologically confirmed cases of scrapie, five of which had congophilic vascular amyloid, were stained immunohistochemically for prion protein (PrP) using a polyclonal antibody. Two clinically suspect but pathologically unconfirmed cases of natural sheep scrapie and the brains of four mice infected with the 111A murine scrapie strain were also examined. Selected sections containing amyloid were stained with each of two peptide antibodies which recognise the N-terminal amino acid residues which are lost following protease digestion of the disease-specific isoform of PrP. The mice infected with the 111A murine scrapie strain had large numbers of hypermature plaques. All the amyloid plaques from both natural sheep scrapie brains and experimental murine brains were heavily immunostained by the polyclonal and both peptide antibodies. In addition, disease-specific accumulations of PrP were detected in endothelial cells or in the intima of blood vessels of the cerebral cortex of sheep scrapie brains. The affected blood vessels were located in areas which otherwise lacked typical scrapie pathology. Vascular accumulations of PrP were also found in leptomeningeal and choroid plexus blood vessels. Vascular amyloid was found mainly in the neocortex. Vascular amyloid and disease-specific parenchymal accumulations of PrP were found in two sheep which showed clinical signs of scrapie but lacked its typical vacuolar pathology. These results show that the mature amyloid of scrapie is composed of, or contains a substantial proportion of, whole length PrP protein. Thus truncation of PrP is not essential for the aggregation of PrP into amyloid. The vascular amyloid of natural sheep scrapie originates from the accumulation and release of PrP from endothelial cells presumably following systemic scrapie infection. The topography of vascular amyloid distribution in Great Britain differs from that reported in the Netherlands. As amyloid deposition in mice is largely controlled by the strain of the infecting agent it is possible that the strain of the agent may influence vascular amyloid deposition. |
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Authors:
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M Jeffrey; C M Goodsir; A Holliman; R J Higgins; M E Bruce; P A McBride; J R Fraser |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: The Veterinary record Volume: 142 ISSN: 0042-4900 ISO Abbreviation: Vet. Rec. Publication Date: 1998 May |
Date Detail:
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Created Date: 1998-08-21 Completed Date: 1998-08-21 Revised Date: 2003-11-14 |
Medline Journal Info:
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Nlm Unique ID: 0031164 Medline TA: Vet Rec Country: ENGLAND |
Other Details:
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Languages: eng Pagination: 534-7 Citation Subset: IM |
Affiliation:
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Lasswade Veterinary Laboratory, Bush Estate, Penicuik, Midlothian. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Amyloid
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analysis*,
immunology Animals Antibody Specificity Brain / immunology Immunohistochemistry Mice Peptide Fragments PrPSc Proteins / analysis*, immunology Scrapie / immunology, physiopathology* Sheep |
| Chemical | |
Reg. No./Substance:
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0/Amyloid; 0/Peptide Fragments; 0/PrPSc Proteins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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