Gabra1+/-/Gabrg2+/- mouse model characterization

Validation Score: 0.900 Price: $0.50 epilepsy with optic atrophy Gabra1+/-/Gabrg2+/- mice Status: proposed

What This Experiment Tests

Validation experiment designed to validate causal mechanisms targeting Gabra1, Gabrg2 in Gabra1+/-/Gabrg2+/- mice. Primary outcome: seizure frequency and visual function

Description

Comprehensive phenotypic characterization of double heterozygous knockout mice (Gabra1+/-/Gabrg2+/-) to model the human microdeletion. The study evaluated spontaneous seizure activity, GABAA receptor expression patterns across brain regions using biochemical and imaging approaches, visual function through electroretinography and visual evoked potentials, and optic nerve structure via electron microscopy. This mouse model recapitulated the human phenotype of epilepsy and visual impairment.

TARGET GENE
Gabra1, Gabrg2
MODEL SYSTEM
Gabra1+/-/Gabrg2+/- mice
ESTIMATED COST
$0
TIMELINE
0 months
PATHWAY
GABAergic neurotransmission
SOURCE
extracted_from_pmid_37703949
PRIMARY OUTCOME
seizure frequency and visual function

Scoring Dimensions

Info Gain 0.00 (25%) Feasibility 0.00 (20%) Hyp Coverage 0.00 (20%) Cost Effect. 0.00 (15%) Novelty 0.00 (10%) Ethical Safety 0.00 (10%) 0.900 composite

📖 Wiki Pages

GABRG2 Protein — GABA-A Receptor Gamma2 SubunitproteinGABRA1 GenegeneGABRG2 — GABA-A Receptor Gamma2 SubunitgeneGABRA1 ProteinproteinBrain RegionsindexEpilepsydiseaseEpilepsydiseaseGABA-A Gamma2 Neuronscell

Protocol

EEG monitoring, electroretinography, visual evoked potentials, electron microscopy, immunohistochemistry

Expected Outcomes

Spontaneous seizures, reduced GABAA receptor expression, optic nerve degeneration

Success Criteria

Recapitulation of human phenotype

Related Hypotheses (1)

Biorhythmic Interference via Controlled Sleep Oscillations0.661

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