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rdf:type |
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lifeskim:mentions |
umls-concept:C0001898,
umls-concept:C0002563,
umls-concept:C0010453,
umls-concept:C0017262,
umls-concept:C0026609,
umls-concept:C0026882,
umls-concept:C0051405,
umls-concept:C0072899,
umls-concept:C0185117,
umls-concept:C0542341,
umls-concept:C0600688,
umls-concept:C1704740,
umls-concept:C1711351,
umls-concept:C2911684
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pubmed:issue |
2
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pubmed:dateCreated |
2004-3-9
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pubmed:abstractText |
The cause of the selective degeneration of motor neurons in amyotrophic lateral sclerosis (ALS) remains a mystery. One potential pathogenic mechanism is excitotoxicity due to disturbances of glutamatergic neurotransmission, particularly via AMPA-sensitive glutamate receptors. We report here that motor neurons from a familial ALS-linked superoxide dismutase (SOD1) mutant G93A mouse show an higher susceptibility to kainate-induced excitotoxicity. Moreover, they expressed GluR(3) and GluR(4) mRNA at detectable levels more frequently, with a modified electrophysiology when compared with control and wild-type SOD1 motor neurons. Thus, the SOD1 G93A mutation causes changes in the AMPA-receptor expression and function, as well as a susceptibility to kainate-mediated excitotoxicity, which may promote the motor neuron degeneration seen in ALS.
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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 |
http://linkedlifedata.com/resource/pubmed/chemical/Alanine,
http://linkedlifedata.com/resource/pubmed/chemical/Glycine,
http://linkedlifedata.com/resource/pubmed/chemical/Kainic Acid,
http://linkedlifedata.com/resource/pubmed/chemical/Neurotoxins,
http://linkedlifedata.com/resource/pubmed/chemical/Protein Subunits,
http://linkedlifedata.com/resource/pubmed/chemical/RNA, Messenger,
http://linkedlifedata.com/resource/pubmed/chemical/Receptors, AMPA,
http://linkedlifedata.com/resource/pubmed/chemical/Superoxide Dismutase,
http://linkedlifedata.com/resource/pubmed/chemical/glutamate receptor ionotropic...,
http://linkedlifedata.com/resource/pubmed/chemical/glutamate receptor ionotropic...
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pubmed:status |
MEDLINE
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pubmed:month |
Mar
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pubmed:issn |
0969-9961
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
15
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
340-50
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pubmed:dateRevised |
2006-11-15
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pubmed:meshHeading |
pubmed-meshheading:15006704-Alanine,
pubmed-meshheading:15006704-Amyotrophic Lateral Sclerosis,
pubmed-meshheading:15006704-Animals,
pubmed-meshheading:15006704-Cells, Cultured,
pubmed-meshheading:15006704-Drug Resistance,
pubmed-meshheading:15006704-Fetus,
pubmed-meshheading:15006704-Genetic Predisposition to Disease,
pubmed-meshheading:15006704-Glycine,
pubmed-meshheading:15006704-Kainic Acid,
pubmed-meshheading:15006704-Membrane Potentials,
pubmed-meshheading:15006704-Mice,
pubmed-meshheading:15006704-Mice, Transgenic,
pubmed-meshheading:15006704-Motor Neurons,
pubmed-meshheading:15006704-Mutation,
pubmed-meshheading:15006704-Neurotoxins,
pubmed-meshheading:15006704-Protein Subunits,
pubmed-meshheading:15006704-RNA, Messenger,
pubmed-meshheading:15006704-Receptors, AMPA,
pubmed-meshheading:15006704-Superoxide Dismutase,
pubmed-meshheading:15006704-Synaptic Transmission
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pubmed:year |
2004
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pubmed:articleTitle |
Cu/Zn-superoxide dismutase (GLY93-->ALA) mutation alters AMPA receptor subunit expression and function and potentiates kainate-mediated toxicity in motor neurons in culture.
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pubmed:affiliation |
Fondazione Santa Lucia IRCCS, 00179 Rome, Italy.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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