Linked Life Data

20085811

Source:http://linkedlifedata.com/resource/pubmed/id/20085811

Statements in which the resource exists.
SubjectPredicateObjectContext
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pubmed-article:20085811pubmed:abstractTextSpinal muscular atrophy (SMA) is caused by insufficient levels of the survival motor neuron (SMN) protein leading to muscle paralysis and respiratory failure. In mouse, introducing the human SMN2 gene partially rescues Smn(-)(/)(-) embryonic lethality. However current models were either too severe or nearly unaffected precluding convenient drug testing for SMA. We report here new SMN2;Smn(-/-) lines carrying one to four copies of the human SMN2 gene. Mice carrying three SMN2 copies exhibited an intermediate phenotype with delayed appearance of motor defects and developmental breathing disorders reminiscent of those found in severe SMA patients. Although normal at birth, at 7 days of age respiratory rate was decreased and apnea frequency was increased in SMA mice in parallel with the appearance of neuromuscular junction defects in the diaphragm. With median survival of 15 days and postnatal onset of neurodegeneration, these mice could be an important tool for evaluating new therapeutics.lld:pubmed
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pubmed-article:20085811pubmed:copyrightInfoCopyright 2010 Elsevier Inc. All rights reserved.lld:pubmed
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pubmed-article:20085811pubmed:volume38lld:pubmed
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pubmed-article:20085811pubmed:year2010lld:pubmed
pubmed-article:20085811pubmed:articleTitleNeuromuscular defects and breathing disorders in a new mouse model of spinal muscular atrophy.lld:pubmed
pubmed-article:20085811pubmed:affiliationTrophos, Parc Scientifique de Luminy, Luminy Biotech Entreprise, Case 931, 13288 Marseille, France.lld:pubmed
pubmed-article:20085811pubmed:publicationTypeJournal Articlelld:pubmed
pubmed-article:20085811pubmed:publicationTypeResearch Support, Non-U.S. Gov'tlld:pubmed
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