rdf:type |
|
lifeskim:mentions |
|
pubmed:issue |
3
|
pubmed:dateCreated |
2005-3-3
|
pubmed:abstractText |
Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by parkinsonism unresponsive to dopaminergic therapy, cerebellar ataxia, and dysautonomia. Neuropathology shows a characteristic neuronal multisystem degeneration that is associated with widespread oligodendroglial alpha-synuclein (alpha-SYN) inclusions. Presently no animal model completely replicates the specific neuropathology of MSA. Here we investigated the behavioral and pathological features resulting from oligodendroglial alpha-SYN overexpression in transgenic mice exposed to mitochondrial inhibition by 3-nitropropionic acid. In transgenic mice 3-nitropropionic acid induced or augmented motor deficits that were associated with MSA-like pathology including striatonigral degeneration and olivopontocerebellar atrophy. Widespread astrogliosis and microglial activation were also observed in the presence of alpha-SYN in oligodendrocytes. Our results indicate that combined mitochondrial inhibition and overexpression of oligodendroglial alpha-SYN generates a novel model of MSA that may be useful for evaluating both pathogenesis and treatment strategies.
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pubmed:commentsCorrections |
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http://linkedlifedata.com/resource/pubmed/commentcorrection/15743798-9923766
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pubmed:language |
eng
|
pubmed:journal |
|
pubmed:citationSubset |
AIM
|
pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/3-nitropropionic acid,
http://linkedlifedata.com/resource/pubmed/chemical/Antigens, CD,
http://linkedlifedata.com/resource/pubmed/chemical/Antigens, Differentiation...,
http://linkedlifedata.com/resource/pubmed/chemical/CD68 antigen, human,
http://linkedlifedata.com/resource/pubmed/chemical/Nerve Tissue Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Nitro Compounds,
http://linkedlifedata.com/resource/pubmed/chemical/Propionic Acids,
http://linkedlifedata.com/resource/pubmed/chemical/Snca protein, mouse,
http://linkedlifedata.com/resource/pubmed/chemical/Synucleins,
http://linkedlifedata.com/resource/pubmed/chemical/alpha-Synuclein
|
pubmed:status |
MEDLINE
|
pubmed:month |
Mar
|
pubmed:issn |
0002-9440
|
pubmed:author |
|
pubmed:issnType |
Print
|
pubmed:volume |
166
|
pubmed:owner |
NLM
|
pubmed:authorsComplete |
Y
|
pubmed:pagination |
869-76
|
pubmed:dateRevised |
2009-11-18
|
pubmed:meshHeading |
pubmed-meshheading:15743798-Animals,
pubmed-meshheading:15743798-Mice,
pubmed-meshheading:15743798-Movement,
pubmed-meshheading:15743798-Neurons,
pubmed-meshheading:15743798-Nitro Compounds,
pubmed-meshheading:15743798-Propionic Acids,
pubmed-meshheading:15743798-Movement Disorders,
pubmed-meshheading:15743798-Oligodendroglia,
pubmed-meshheading:15743798-Astrocytes,
pubmed-meshheading:15743798-Time Factors,
pubmed-meshheading:15743798-Disease Models, Animal,
pubmed-meshheading:15743798-Nerve Tissue Proteins,
pubmed-meshheading:15743798-Image Processing, Computer-Assisted,
pubmed-meshheading:15743798-Immunohistochemistry,
pubmed-meshheading:15743798-Microglia,
pubmed-meshheading:15743798-Antigens, CD,
pubmed-meshheading:15743798-Antigens, Differentiation, Myelomonocytic
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