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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
3
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pubmed:dateCreated |
2007-5-22
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pubmed:abstractText |
The molecular mechanisms underlying the selective neurodegeneration of motor neurons in amyotrophic lateral sclerosis (ALS) are inadequately understood. Recent breakthroughs have implicated impaired axonal transport, mediated by molecular motors, as a key element for disease onset and progression. The current work identifies the expression of 15 kinesin-like motors in healthy human motor cortex, including three novel isoforms. Our comprehensive quantitative mRNA analysis in control and sporadic ALS (SALS) motor cortex specimens detects SALS-specific down-regulation of KIF1Bbeta and novel KIF3Abeta, two isoforms we show to be enriched in the brain, and also of SOD1, a key enzyme linked to familial ALS. This is accompanied by a marked reduction of KIF3Abeta protein levels. In the motor cortex KIF3Abeta localizes in cholinergic neurons, including upper motor neurons. No mutations causing splicing defects or altering protein-coding sequences were identified in the genes of the three proteins. The present study implicates two motor proteins as possible candidates in SALS pathology.
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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/Genetic Markers,
http://linkedlifedata.com/resource/pubmed/chemical/KIF1B protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/KIF3A protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Kinesin,
http://linkedlifedata.com/resource/pubmed/chemical/Molecular Motor Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Nerve Tissue Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/Protein Isoforms,
http://linkedlifedata.com/resource/pubmed/chemical/RNA, Messenger,
http://linkedlifedata.com/resource/pubmed/chemical/Superoxide Dismutase,
http://linkedlifedata.com/resource/pubmed/chemical/superoxide dismutase 1
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pubmed:status |
MEDLINE
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pubmed:month |
Jun
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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 |
26
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
577-89
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pubmed:meshHeading |
pubmed-meshheading:17418584-Aged,
pubmed-meshheading:17418584-Alternative Splicing,
pubmed-meshheading:17418584-Amyotrophic Lateral Sclerosis,
pubmed-meshheading:17418584-Animals,
pubmed-meshheading:17418584-Cholinergic Fibers,
pubmed-meshheading:17418584-Disease Models, Animal,
pubmed-meshheading:17418584-Down-Regulation,
pubmed-meshheading:17418584-Female,
pubmed-meshheading:17418584-Gene Expression Regulation,
pubmed-meshheading:17418584-Genetic Markers,
pubmed-meshheading:17418584-Humans,
pubmed-meshheading:17418584-Kinesin,
pubmed-meshheading:17418584-Male,
pubmed-meshheading:17418584-Mice,
pubmed-meshheading:17418584-Mice, Transgenic,
pubmed-meshheading:17418584-Middle Aged,
pubmed-meshheading:17418584-Molecular Motor Proteins,
pubmed-meshheading:17418584-Motor Cortex,
pubmed-meshheading:17418584-Motor Neurons,
pubmed-meshheading:17418584-Mutation,
pubmed-meshheading:17418584-Nerve Tissue Proteins,
pubmed-meshheading:17418584-Protein Isoforms,
pubmed-meshheading:17418584-RNA, Messenger,
pubmed-meshheading:17418584-Superoxide Dismutase
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pubmed:year |
2007
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pubmed:articleTitle |
Differential expression of molecular motors in the motor cortex of sporadic ALS.
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pubmed:affiliation |
Department of Biological Sciences, University of Cyprus and Cyprus Institute of Neurology and Genetics, P.O. Box 20537, 1678 Nicosia, Cyprus.
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pubmed:publicationType |
Journal Article,
Research Support, Non-U.S. Gov't
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