Clinical characterization of GABRA1/GABRG2 microdeletion patient

Clinical Score: 0.950 Price: $0.50 epilepsy with optic atrophy human patient Status: proposed

What This Experiment Tests

Clinical experiment designed to assess clinical efficacy targeting GABRA1, GABRG2 in human patient. Primary outcome: clinical phenotype characterization

Description

Clinical evaluation of a human patient with microdeletion of GABRA1 and GABRG2 genes presenting with intractable epilepsy and blindness due to optic atrophy. The study included electroencephalography recordings to characterize seizure activity and fundus photography to evaluate bilateral optic nerve atrophy. This represents the first reported case of haploinsufficiency of both GABR genes causing this phenotype combination.

TARGET GENE
GABRA1, GABRG2
MODEL SYSTEM
human patient
ESTIMATED COST
$0
TIMELINE
0 months
PATHWAY
GABAergic neurotransmission
SOURCE
extracted_from_pmid_37703949
PRIMARY OUTCOME
clinical phenotype characterization

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%) 0.950 composite

📖 Wiki Pages

GABRG2 — GABA-A Receptor Gamma2 SubunitgeneGABRA1 GenegeneGABRG2 Protein — GABA-A Receptor Gamma2 SubunitproteinGABRA1 ProteinproteinEpilepsydiseaseSerotonergic Dysfunction in NeurodegenerationmechanismGABA-A Gamma2 NeuronscellGABA-A Alpha1 NeuronscellFastigial Nucleus (Fastigial Cerebellar Nucleus) NcellGABAergic Signaling Pathway in NeurodegenerationmechanismParvalbumin Neurons (PV+)cellNucleus of the Lateral Lemniscus (LLL) NeuronscellInferior Temporal Cortex Neurons in Prosopagnosiacell

Protocol

Electroencephalography and fundus photography

Expected Outcomes

Seizure activity and optic nerve abnormalities

Success Criteria

Documentation of epilepsy and visual impairment

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