Abstract
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.
Publication types
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Comparative Study
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Research Support, Non-U.S. Gov't
MeSH terms
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Aged
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Amyotrophic Lateral Sclerosis / genetics*
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Amyotrophic Lateral Sclerosis / pathology
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Animals
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Animals, Genetically Modified
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Cell Death / genetics*
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Humans
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Male
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Mice
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Motor Neurons / pathology
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Motor Neurons / physiology*
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Muscular Atrophy, Spinal / genetics*
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Muscular Atrophy, Spinal / pathology
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Mutation
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RNA Editing
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RNA, Messenger
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Receptors, AMPA / genetics*
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Reverse Transcriptase Polymerase Chain Reaction
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Spinal Cord / pathology
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Superoxide Dismutase / genetics
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Superoxide Dismutase-1
Substances
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RNA, Messenger
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Receptors, AMPA
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SOD1 protein, human
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Sod1 protein, mouse
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Sod1 protein, rat
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Superoxide Dismutase
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Superoxide Dismutase-1
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glutamate receptor ionotropic, AMPA 2