pubmed-article:12084935 | rdf:type | pubmed:Citation | lld:pubmed |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C0086418 | lld:lifeskim |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C0025936 | lld:lifeskim |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C0241888 | lld:lifeskim |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C0015127 | lld:lifeskim |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C0524851 | lld:lifeskim |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C0026882 | lld:lifeskim |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C0040005 | lld:lifeskim |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C0285890 | lld:lifeskim |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C1314792 | lld:lifeskim |
pubmed-article:12084935 | lifeskim:mentions | umls-concept:C0332621 | lld:lifeskim |
pubmed-article:12084935 | pubmed:issue | 13 | lld:pubmed |
pubmed-article:12084935 | pubmed:dateCreated | 2002-6-26 | lld:pubmed |
pubmed-article:12084935 | pubmed:abstractText | Mutations in alpha-synuclein (alpha-Syn) cause Parkinson's disease (PD) in a small number of pedigrees with familial PD. Moreover, alpha-Syn accumulates as a major component of Lewy bodies and Lewy neurites, intraneuronal inclusions that are neuropathological hallmarks of PD. To better understand the pathogenic relationship between alterations in the biology of alpha-Syn and PD-associated neurodegeneration, we generated multiple lines of transgenic mice expressing high levels of either wild-type or familial PD-linked Ala-30 --> Pro (A30P) or Ala-53 --> Thr (A53T) human alpha-Syns. The mice expressing the A53T human alpha-Syn, but not wild-type or the A30P variants, develop adult-onset neurodegenerative disease with a progressive motoric dysfunction leading to death. Pathologically, affected mice exhibit neuronal abnormalities (in perikarya and neurites) including pathological accumulations of alpha-Syn and ubiquitin. Consistent with abnormal neuronal accumulation of alpha-Syn, brain regions with pathology exhibit increases in detergent-insoluble alpha-Syn and alpha-Syn aggregates. Our results demonstrate that the A53T mutant alpha-Syn causes significantly greater in vivo neurotoxicity as compared with other alpha-Syn variants. Further, alpha-Syn-dependent neurodegeneration is associated with abnormal accumulation of detergent-insoluble alpha-Syn. | lld:pubmed |
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pubmed-article:12084935 | pubmed:language | eng | lld:pubmed |
pubmed-article:12084935 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
pubmed-article:12084935 | pubmed:citationSubset | IM | lld:pubmed |
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pubmed-article:12084935 | pubmed:status | MEDLINE | lld:pubmed |
pubmed-article:12084935 | pubmed:month | Jun | lld:pubmed |
pubmed-article:12084935 | pubmed:issn | 0027-8424 | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:JenkinsNancy... | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:CopelandNeal... | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:LeeMichael... | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:DawsonTed MTM | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:StirlingWanda... | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:XuYanqunY | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:XuXueyingX | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:QuiDikeD | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:MandirAllen... | lld:pubmed |
pubmed-article:12084935 | pubmed:author | pubmed-author:PriceDon LDL | lld:pubmed |
pubmed-article:12084935 | pubmed:issnType | Print | lld:pubmed |
pubmed-article:12084935 | pubmed:day | 25 | lld:pubmed |
pubmed-article:12084935 | pubmed:volume | 99 | lld:pubmed |
pubmed-article:12084935 | pubmed:owner | NLM | lld:pubmed |
pubmed-article:12084935 | pubmed:authorsComplete | Y | lld:pubmed |
pubmed-article:12084935 | pubmed:pagination | 8968-73 | lld:pubmed |
pubmed-article:12084935 | pubmed:dateRevised | 2009-11-18 | lld:pubmed |
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pubmed-article:12084935 | pubmed:meshHeading | pubmed-meshheading:12084935... | lld:pubmed |
pubmed-article:12084935 | pubmed:year | 2002 | lld:pubmed |
pubmed-article:12084935 | pubmed:articleTitle | Human alpha-synuclein-harboring familial Parkinson's disease-linked Ala-53 --> Thr mutation causes neurodegenerative disease with alpha-synuclein aggregation in transgenic mice. | lld:pubmed |
pubmed-article:12084935 | pubmed:affiliation | Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21205-2196, USA. mklee@jhmi.edu | lld:pubmed |