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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
15
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pubmed:dateCreated |
2002-7-24
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pubmed:abstractText |
Neuronal degeneration in spinal muscular atrophy is caused by reduced expression of the survival motor neuron (SMN) protein. SMN and the tightly interacting Gemin2 form part of a macromolecular complex (SMN complex) that mediates assembly of spliceosomal small nuclear ribonucleoproteins (U snRNPs). We used mouse genetics to investigate the function of this complex in motoneuron maintenance. Reduced Smn/Gemin2 protein levels lead to disturbed U snRNP assembly as indicated by reduced nuclear accumulation of Sm proteins. This finding correlates with enhanced motoneuron degeneration in Gemin2(+/-)/Smn(+/-) mice. Our data provide in vivo evidence that impaired production of U snRNPs contributes to motoneuron degeneration.
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10339583,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10486205,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10500148,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10545592,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10556282,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10601333,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10655541,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10655542,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10679938,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10725331,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10749973,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10767334,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10816558,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10909848,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-10942426,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-11113446,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-11181573,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-11343899,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-11354640,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-11689659,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-11714716,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-11715014,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-11748230,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-6179522,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-7813012,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-8231868,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-8361533,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-8614490,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-8670859,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-9275227,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-9323129,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-9323130,
http://linkedlifedata.com/resource/pubmed/commentcorrection/12091709-9845364
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Jul
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pubmed:issn |
0027-8424
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
23
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pubmed:volume |
99
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
10126-31
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:12091709-Animals,
pubmed-meshheading:12091709-Cell Death,
pubmed-meshheading:12091709-Cyclic AMP Response Element-Binding Protein,
pubmed-meshheading:12091709-Disease Models, Animal,
pubmed-meshheading:12091709-Gene Targeting,
pubmed-meshheading:12091709-Mice,
pubmed-meshheading:12091709-Mice, Knockout,
pubmed-meshheading:12091709-Motor Neurons,
pubmed-meshheading:12091709-Muscular Atrophy, Spinal,
pubmed-meshheading:12091709-Nerve Tissue Proteins,
pubmed-meshheading:12091709-RNA-Binding Proteins,
pubmed-meshheading:12091709-Restriction Mapping,
pubmed-meshheading:12091709-Ribonucleoproteins, Small Nuclear,
pubmed-meshheading:12091709-SMN Complex Proteins
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pubmed:year |
2002
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pubmed:articleTitle |
Gene targeting of Gemin2 in mice reveals a correlation between defects in the biogenesis of U snRNPs and motoneuron cell death.
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pubmed:affiliation |
Institute of Clinical Neurobiology, Josef-Schneider Strasse 11, D-97080 Würzburg, Germany.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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