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Molecular mechanism underlying death of motor neurons in sporadic ALS: towards specific therapy Shin Kwak 1 1Department of Neurology, Graduate School of Medicine, The University of Tokyo Keyword: amyotrophic lateral sclerosis , AMPA receptor , GluR2 , neuronal death , RNA editing pp.902-911
Published Date 2006/12/10
DOI https://doi.org/10.11477/mf.1431100411
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 AMPA receptor-mediated neuronal death plays a pivotal role in motor neuron diseases, and an increase of Ca2+influx has been proposed to initiate the death cascade. Both a decrease of RNA editing at the GluR2 Q/R site and a decrease of relative GluR2 level among AMPA receptor subunits result in an increase of Ca2+-permeable AMPA receptors expressed on neurons. The former mechanism plays in sporadic amyotrophic lateral sclerosis(ALS), whereas the latter is the mechanism underlying familial ALS(ALS1)linked to mutated cupper-zinc superoxide dismutase gene(SOD1). On the other hand, AMPA receptor-mediated mechanism does not seem to play any role in death of motor neurons in X-linked spinal and bulbar muscular atrophy(SBMA). The difference of molecular mechanism underlying neuronal death in sporadic ALS and ALS1 implies that the effective therapy to ALS1 may not be applicable to sporadic ALS and a specific strategy may be necessary to develop specific therapy for sporadic ALS.


Copyright © 2006, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1243 印刷版ISSN 0001-8724 医学書院

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