Source:http://linkedlifedata.com/resource/pubmed/id/15091340
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
2
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pubmed:dateCreated |
2004-4-19
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pubmed:abstractText |
Cysteine string protein alpha (CSPalpha)--an abundant synaptic vesicle protein that contains a DNA-J domain characteristic of Hsp40 chaperones--is thought to regulate Ca2+ channels and/or synaptic vesicle exocytosis. We now show that, in young mice, deletion of CSPalpha does not impair survival and causes no significant changes in presynaptic Ca2+ currents or synaptic vesicle exocytosis as measured in the Calyx of Held synapse. At 2-4 weeks of age, however, CSPalpha-deficient mice develop a progressive, fatal sensorimotor disorder. The neuromuscular junctions and Calyx synapses of CSPalpha-deficient mice exhibit increasing neurodegenerative changes, synaptic transmission becomes severely impaired, and the mutant mice die at approximately 2 months of age. Our data suggest that CSPalpha is not essential for the normal operation of Ca2+ channels or exocytosis but acts as a presynaptic chaperone that maintains continued synaptic function, raising the possibility that enhanced CSPalpha function could attenuate neurodegenerative diseases.
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pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Apr
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pubmed:issn |
0896-6273
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pubmed:author |
pubmed-author:Castellano-MuñozManuelM,
pubmed-author:Fernández-ChacónRafaelR,
pubmed-author:MontesinosMaria LML,
pubmed-author:NishimuneHiroshiH,
pubmed-author:RosenmundChristianC,
pubmed-author:SüdhofThomas CTC,
pubmed-author:SanesJoshua RJR,
pubmed-author:SchmitzFrankF,
pubmed-author:SchneggenburgerRalfR,
pubmed-author:TabaresLuciaL,
pubmed-author:WölfelMarkusM
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pubmed:issnType |
Print
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pubmed:day |
22
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pubmed:volume |
42
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
237-51
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:15091340-Animals,
pubmed-meshheading:15091340-Animals, Newborn,
pubmed-meshheading:15091340-Brain,
pubmed-meshheading:15091340-HSP40 Heat-Shock Proteins,
pubmed-meshheading:15091340-Membrane Proteins,
pubmed-meshheading:15091340-Mice,
pubmed-meshheading:15091340-Mice, Knockout,
pubmed-meshheading:15091340-Nerve Degeneration,
pubmed-meshheading:15091340-Neuromuscular Junction,
pubmed-meshheading:15091340-Presynaptic Terminals,
pubmed-meshheading:15091340-Synaptic Vesicles
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pubmed:year |
2004
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pubmed:articleTitle |
The synaptic vesicle protein CSP alpha prevents presynaptic degeneration.
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pubmed:affiliation |
Center for Basic Neuroscience, Department of Molecular Genetics and Howard Hughes Medical Institute, University of Texas Southwestern Medical Center, Dallas 75390, USA.
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pubmed:publicationType |
Journal Article,
Comparative Study,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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