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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
5642
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pubmed:dateCreated |
2003-10-3
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pubmed:abstractText |
The most common inherited [correct] form of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting adult motor neurons, is caused by dominant mutations in the ubiquitously expressed Cu-Zn superoxide dismutase (SOD1). In chimeric mice that are mixtures of normal and SOD1 mutant-expressing cells, toxicity to motor neurons is shown to require damage from mutant SOD1 acting within nonneuronal cells. Normal motor neurons in SOD1 mutant chimeras develop aspects of ALS pathology. Most important, nonneuronal cells that do not express mutant SOD1 delay degeneration and significantly extend survival of mutant-expressing motor neurons.
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pubmed:grant |
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pubmed:commentsCorrections |
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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 |
Oct
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pubmed:issn |
1095-9203
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pubmed:author |
pubmed-author:BoilléeSS,
pubmed-author:BrownR HRHJr,
pubmed-author:ClemensA LAL,
pubmed-author:ClevelandD WDW,
pubmed-author:DoucetteWW,
pubmed-author:FerranteR JRJ,
pubmed-author:GarciaM LML,
pubmed-author:GoldsteinL S BLS,
pubmed-author:JulienJ-PJP,
pubmed-author:McMahonA PAP,
pubmed-author:NguyenM DMD,
pubmed-author:RobertsE AEA,
pubmed-author:RuleMM,
pubmed-author:SiwekDD
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pubmed:issnType |
Electronic
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pubmed:day |
3
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pubmed:volume |
302
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
113-7
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pubmed:dateRevised |
2007-11-14
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pubmed:meshHeading |
pubmed-meshheading:14526083-Amyotrophic Lateral Sclerosis,
pubmed-meshheading:14526083-Animals,
pubmed-meshheading:14526083-Axons,
pubmed-meshheading:14526083-Cell Survival,
pubmed-meshheading:14526083-Chimera,
pubmed-meshheading:14526083-Humans,
pubmed-meshheading:14526083-Mice,
pubmed-meshheading:14526083-Mice, Inbred C57BL,
pubmed-meshheading:14526083-Mice, Inbred Strains,
pubmed-meshheading:14526083-Mice, Transgenic,
pubmed-meshheading:14526083-Motor Neurons,
pubmed-meshheading:14526083-Mutation,
pubmed-meshheading:14526083-Nerve Degeneration,
pubmed-meshheading:14526083-Neurofilament Proteins,
pubmed-meshheading:14526083-Spinal Cord,
pubmed-meshheading:14526083-Superoxide Dismutase,
pubmed-meshheading:14526083-Survival Rate,
pubmed-meshheading:14526083-Ubiquitin
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pubmed:year |
2003
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pubmed:articleTitle |
Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice.
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pubmed:affiliation |
Ludwig Institute for Cancer Research, University of California, 9500 Gilman Drive, La Jolla, CA 92093-0670, USA.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, U.S. Gov't, Non-P.H.S.,
Research Support, Non-U.S. Gov't
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