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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
1-2
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pubmed:dateCreated |
2006-11-28
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pubmed:abstractText |
ALS is a devastating neurodegenerative disorder for which no effective treatment exists. The precise molecular mechanisms underlying the selective degeneration of motor neurons are still unknown. A motor neuron specific apoptotic pathway involving Fas and NO has been discovered. Motor neurons from ALS-mice have an increased sensitivity to Fas-induced cell death via this pathway. In this study we therefore crossed G93A-SOD1 overexpressing ALS mice with Fas ligand (FasL) mutant (gld) mice to investigate whether the reduced Fas signaling could have beneficial effects on motor neuron death. G93A-SOD1 mutant mice with a homozygous FasL mutant showed a modest but statistically significant extension of survival, and reduced loss of motor neurons. These results indicate that motor neuron apoptosis triggered by Fas is relevant in ALS pathogenesis.
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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 |
Dec
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pubmed:issn |
0022-510X
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
21
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pubmed:volume |
251
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
44-9
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pubmed:meshHeading |
pubmed-meshheading:17049562-Age Factors,
pubmed-meshheading:17049562-Amyotrophic Lateral Sclerosis,
pubmed-meshheading:17049562-Animals,
pubmed-meshheading:17049562-Behavior, Animal,
pubmed-meshheading:17049562-Cell Count,
pubmed-meshheading:17049562-Disease Models, Animal,
pubmed-meshheading:17049562-Fas Ligand Protein,
pubmed-meshheading:17049562-Immunohistochemistry,
pubmed-meshheading:17049562-Mice,
pubmed-meshheading:17049562-Mice, Inbred C57BL,
pubmed-meshheading:17049562-Mice, Transgenic,
pubmed-meshheading:17049562-Motor Activity,
pubmed-meshheading:17049562-Motor Neurons,
pubmed-meshheading:17049562-Nitric Oxide Synthase Type I,
pubmed-meshheading:17049562-Probability,
pubmed-meshheading:17049562-Spinal Cord,
pubmed-meshheading:17049562-Superoxide Dismutase,
pubmed-meshheading:17049562-Survival Analysis,
pubmed-meshheading:17049562-Tyrosine
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pubmed:year |
2006
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pubmed:articleTitle |
Loss of Fas ligand-function improves survival in G93A-transgenic ALS mice.
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pubmed:affiliation |
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York-Presbyterian Hospital, New York, NY, USA. petri.susanne@mh-hannover.de
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't,
Research Support, N.I.H., Extramural
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