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- W2363570682 abstract "α-amino-3-hydroxy-5-methl-4-isoxazolepropionic acid(AMPA) receptor,one of ionotropic glutamate receptors,has been proposed to play a critical role to initiate the neuronal death cascade in motor neuron disease by an increase of Ca~(2+) influx.These are at least two mechanisms to increase Ca~(2+) influx through Ca~(2+)-permiable AMPA receptor:a decrease of RNA editing efficiency at the GluR2 Q/R site and a decrease of GluR2 level relative to AMPA receptor submits.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).On the other hand,relative low GluR2 level among AMPA receptor subunits seems to increase Ca~(2+) permeability of motor neurons in familial ALS(ALS1) linked to mutated cupper-zinc superoxide dismutase gene(SOD1).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).From the molecular pathomechanism of sporadic ALS and ALS1,drugs which increase RNA editing efficacy at the GluR2 Q/R site could be a potent therapy for sporadic ALS,while AMPA receptor antagonists could prevent deterioration from ALS1." @default.
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- W2363570682 date "2009-01-01" @default.
- W2363570682 modified "2023-10-01" @default.
- W2363570682 title "Amyotropic Lateral Sclerosis and Excitatory Amino Acid" @default.
- W2363570682 hasPublicationYear "2009" @default.
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