| pubmed-article:16597650 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:16597650 | lifeskim:mentions | umls-concept:C0033164 | lld:lifeskim |
| pubmed-article:16597650 | lifeskim:mentions | umls-concept:C0439660 | lld:lifeskim |
| pubmed-article:16597650 | lifeskim:mentions | umls-concept:C0162534 | lld:lifeskim |
| pubmed-article:16597650 | lifeskim:mentions | umls-concept:C0332281 | lld:lifeskim |
| pubmed-article:16597650 | lifeskim:mentions | umls-concept:C1852220 | lld:lifeskim |
| pubmed-article:16597650 | lifeskim:mentions | umls-concept:C0596988 | lld:lifeskim |
| pubmed-article:16597650 | lifeskim:mentions | umls-concept:C0443199 | lld:lifeskim |
| pubmed-article:16597650 | pubmed:issue | Pt 6 | lld:pubmed |
| pubmed-article:16597650 | pubmed:dateCreated | 2006-6-1 | lld:pubmed |
| pubmed-article:16597650 | pubmed:abstractText | Inherited prion diseases are caused by PRNP coding mutations and display marked phenotypic heterogeneity within families segregating the same pathogenic mutation. A proline-to-leucine substitution at prion protein (PrP) residue 102 (P102L), classically associated with the Gerstmann-Sträussler-Scheinker (GSS) phenotype, also shows marked clinical and pathological heterogeneity, including patients with a Creutzfeldt-Jakob disease (CJD) phenotype. To date, this heterogeneity has been attributed to temporal and spatial variance in the propagation of distinct protease-resistant (PrP(Sc)) isoforms of mutant PrP. Here, using a monoclonal antibody that recognizes wild-type PrP, but not PrP 102L, we reveal a spectrum of involvement of wild-type PrP(Sc) in P102L individuals. PrP(Sc) isoforms derived from wild-type and mutant PrP are distinct both from each other and from those seen in sporadic and acquired CJD. Such differential propagation of disease-related isoforms of wild-type PrP and PrP 102L provides a molecular mechanism for generation of the multiple clinicopathological phenotypes seen in inherited prion disease. | lld:pubmed |
| pubmed-article:16597650 | pubmed:language | eng | lld:pubmed |
| pubmed-article:16597650 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16597650 | pubmed:citationSubset | AIM | lld:pubmed |
| pubmed-article:16597650 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16597650 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16597650 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16597650 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16597650 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:16597650 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:16597650 | pubmed:month | Jun | lld:pubmed |
| pubmed-article:16597650 | pubmed:issn | 1460-2156 | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:BudkaHerbertH | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:BrandnerSebas... | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:CollingeJohnJ | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:WadsworthJona... | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:AsanteEmmanue... | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:LinehanJacque... | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:JoinerSusanS | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:GowlandIanI | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:CooperSharonS | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:MallinsonGary... | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:BuckellJennif... | lld:pubmed |
| pubmed-article:16597650 | pubmed:author | pubmed-author:PowellCarolin... | lld:pubmed |
| pubmed-article:16597650 | pubmed:issnType | Electronic | lld:pubmed |
| pubmed-article:16597650 | pubmed:volume | 129 | lld:pubmed |
| pubmed-article:16597650 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:16597650 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:16597650 | pubmed:pagination | 1557-69 | lld:pubmed |
| pubmed-article:16597650 | pubmed:dateRevised | 2006-11-15 | lld:pubmed |
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| pubmed-article:16597650 | pubmed:meshHeading | pubmed-meshheading:16597650... | lld:pubmed |
| pubmed-article:16597650 | pubmed:meshHeading | pubmed-meshheading:16597650... | lld:pubmed |
| pubmed-article:16597650 | pubmed:meshHeading | pubmed-meshheading:16597650... | lld:pubmed |
| pubmed-article:16597650 | pubmed:meshHeading | pubmed-meshheading:16597650... | lld:pubmed |
| pubmed-article:16597650 | pubmed:meshHeading | pubmed-meshheading:16597650... | lld:pubmed |
| pubmed-article:16597650 | pubmed:year | 2006 | lld:pubmed |
| pubmed-article:16597650 | pubmed:articleTitle | Phenotypic heterogeneity in inherited prion disease (P102L) is associated with differential propagation of protease-resistant wild-type and mutant prion protein. | lld:pubmed |
| pubmed-article:16597650 | pubmed:affiliation | MRC Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London, National Hospital for Neurology and Neurosurgery Queen Square, London, UK. | lld:pubmed |
| pubmed-article:16597650 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:16597650 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
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