Early-onset epilepsy and postnatal lethality associated with an editing-deficient GluR-B allele in mice.

Authors: Brusa, R  Zimmermann, F  Koh, DS  Feldmeyer, D  Gass, P  Seeburg, PH  Sprengel, R 
Citation: Brusa R, etal., Science 1995 Dec 8;270(5242):1677-80.
Pubmed: (View Article at PubMed) PMID:7502080

The arginine residue at position 586 of the GluR-B subunit renders heteromeric alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionate (AMPA)-sensitive glutamate receptor channels impermeable to calcium. The codon for this arginine is introduced at the precursor messenger RNA (pre-mRNA) stage by site-selective adenosine editing of a glutamine codon. Heterozygous mice engineered by gene targeting to harbor an editing-incompetent GluR-B allele synthesized unedited GluR-B subunits and, in principal neurons and interneurons, expressed AMPA receptors with increased calcium permeability. These mice developed seizures and died by 3 weeks of age, showing that GluR-B pre-mRNA editing is essential for brain function.

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CRRD ID: 737715
Created: 2004-02-26
Species: All species
Last Modified: 2004-02-26
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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.