Journal
NEUROSCIENCE RESEARCH
Volume 54, Issue 1, Pages 11-14Publisher
ELSEVIER IRELAND LTD
DOI: 10.1016/j.neures.2005.09.006
Keywords
ALS; SOD1; spinal and bulbar muscular atrophy; motor neuron; RNA editing; GluR2; AMPA receptor; neuronal death
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Deficient RNA editing of the AMPA receptor subunit GluR2 at the Q/R. site is a primary cause of neuronal death and recently has been reported to be a tightly linked etiological cause of motor neuron death in sporadic amyotrophic lateral sclerosis (ALS). We quantified the RNA editing efficiency of the GluR2 Q/R site in single motor neurons of rats transgenic for mutant human Cu/Zn-superoxide dismutase (SOD1) as well as patients with spinal and bulbar muscular atrophy (SBMA), and found that GluR2 mRNA was completely edited in all the motor neurons examined. It seems likely that the death cascade is different among the dying motor neurons in sporadic ALS, familial ALS with mutant SOD1 and SBMA. (c) 2005 Elsevier Ireland Ltd and the Japan Neuroscience Society. All rights reserved.
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