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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Gabrg2+
wild type
MGI:2430723
Summary 9 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
ht1
 		Gabrg2tm1.1Stmo/Gabrg2+ B6.129S2-Gabrg2tm1.1Stmo MGI:4415072
ht2
 		Gabrg2em1(IMPC)Mbp/Gabrg2+ C57BL/6NCrl-Gabrg2em1(IMPC)Mbp/MbpMmucd MGI:7414901
ht3
 		Gabrg2tm1Spet/Gabrg2+ involves: 129 * BALB/cJ * C57BL/6 * DBA/2J MGI:3764958
ht4
 		Gabrg2tm2Spet/Gabrg2+ involves: 129S1/Sv * 129X1/SvJ MGI:5474681
ht5
 		Gabrg2tm1Spet/Gabrg2+ involves: 129S1/Sv * 129X1/SvJ * BALB/cJ MGI:5474682
ht6
 		Gabrg2tm1Spet/Gabrg2+ involves: 129S1/Sv * 129X1/SvJ * BALB/cJ * C57BL/6 MGI:3764957
ht7
 		Gabrg2tm1Geh/Gabrg2+ involves: 129S1/Sv * 129X1/SvJ * C57BL/6J MGI:3835048
ht8
 		Gabrg2tm1.2Rmac/Gabrg2+ involves: C57BL/6 * C57BL/6J * SJL MGI:6740192
cn9
 		Gabrg2tm2Spet/Gabrg2+
Tg(Camk2a-tTA)1Mmay/0
Tg(tetO-cre)LC1Bjd/0 		involves: 129S1/Sv * 129X1/SvJ * BALB/c * C57BL/6 MGI:5474679


Genotype
MGI:4415072
ht1
Allelic
Composition		 Gabrg2tm1.1Stmo/Gabrg2+
Genetic
Background		 B6.129S2-Gabrg2tm1.1Stmo
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gabrg2tm1.1Stmo mutation (0 available); any Gabrg2 mutation (40 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
behavior/neurological
behavior/neurological phenotype ( J:154745 )
N
• mice exhibit normal exploratory, locomotor, and anxiety-related behaviors
abnormal spatial reference memory ( J:154745 )
• mice exhibit deficits in spatial object recognition compared with wild-type mice
• however, mice exhibit normal object recognition memory

nervous system
abnormal synapse morphology ( J:154745 )
• the size of the inhibitory synapses in the CA3 region are increased compared to in wild-type mice
• however, synapses in the CA1 region are normal
abnormal miniature inhibitory postsynaptic currents ( J:154745 )
• in the CA3 hippocampal neurons, miniature inhibitory postsynaptic current (mIPSC) amplitude and frequency are increased compared to in wild-type mice
• however, mIPSCs in CA1 neurons are normal




Genotype
MGI:7414901
ht2
Allelic
Composition		 Gabrg2em1(IMPC)Mbp/Gabrg2+
Genetic
Background		 C57BL/6NCrl-Gabrg2em1(IMPC)Mbp/MbpMmucd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gabrg2em1(IMPC)Mbp mutation (1 available); any Gabrg2 mutation (40 available)
Data Sources
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
cardiovascular system

endocrine/exocrine glands
enlarged testis ( J:211773 )
IMPC - UCD

integument

reproductive system
enlarged testis ( J:211773 )
IMPC - UCD

skeleton

vision/eye




Genotype
MGI:3764958
ht3
Allelic
Composition		 Gabrg2tm1Spet/Gabrg2+
Genetic
Background		 involves: 129 * BALB/cJ * C57BL/6 * DBA/2J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gabrg2tm1Spet mutation (3 available); any Gabrg2 mutation (40 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
absence seizures ( J:127120 )
• Background Sensitivity: more mice (greater than 90%) exhibit symptomatic spike-wave discharge (SWD) on a DBA/2J background than on a C57BL/6 background with longer SWD episodes (2.53+/-0.21 ms compared to 1.20+/-0.16 ms on a C57BL/6 background)
• Background Sensitivity: paroxysmal discharges are first noted at P20, by P22 amplitudes of the sharp waves are increased with associated behavioral arrest and frank epileptiform discharges occur by P24 to P28 with distinct spikes and wave morphology associated with behavioral arrest
• treatment with ethosuximide (200 mg per kg) reduces absence seizure
abnormal inhibitory postsynaptic currents ( J:127120 )
• miniature inhibitory postsynaptic current amplitudes of cortical neurons are lower than in wild-type mice (57.9+/-2.9 pA compared to 66.4+/-1.8 pA in wild-type mice)
• however, spontaneous inhibitory postsynaptic currents in the thalamic reticular nuclei are normal

behavior/neurological
absence seizures ( J:127120 )
• Background Sensitivity: more mice (greater than 90%) exhibit symptomatic spike-wave discharge (SWD) on a DBA/2J background than on a C57BL/6 background with longer SWD episodes (2.53+/-0.21 ms compared to 1.20+/-0.16 ms on a C57BL/6 background)
• Background Sensitivity: paroxysmal discharges are first noted at P20, by P22 amplitudes of the sharp waves are increased with associated behavioral arrest and frank epileptiform discharges occur by P24 to P28 with distinct spikes and wave morphology associated with behavioral arrest
• treatment with ethosuximide (200 mg per kg) reduces absence seizure




Genotype
MGI:5474681
ht4
Allelic
Composition		 Gabrg2tm2Spet/Gabrg2+
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gabrg2tm2Spet mutation (0 available); any Gabrg2 mutation (40 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
behavior/neurological
decreased susceptibility to pharmacologically induced seizures ( J:194196 )
• induced by pentylenetetrazil compared with Gabrg2tm2Spet/Gabrg2+ Tg(Camk2a-tTA)1Mmay Tg(tetO-cre)LC1Bjd mice (no doxycycline treatment)
• however, no seizure kindling effect is observed

nervous system
decreased susceptibility to pharmacologically induced seizures ( J:194196 )
• induced by pentylenetetrazil compared with Gabrg2tm2Spet/Gabrg2+ Tg(Camk2a-tTA)1Mmay Tg(tetO-cre)LC1Bjd mice (no doxycycline treatment)
• however, no seizure kindling effect is observed




Genotype
MGI:5474682
ht5
Allelic
Composition		 Gabrg2tm1Spet/Gabrg2+
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * BALB/cJ
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gabrg2tm1Spet mutation (3 available); any Gabrg2 mutation (40 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
behavior/neurological

nervous system




Genotype
MGI:3764957
ht6
Allelic
Composition		 Gabrg2tm1Spet/Gabrg2+
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * BALB/cJ * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gabrg2tm1Spet mutation (3 available); any Gabrg2 mutation (40 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
nervous system
increased susceptibility to pharmacologically induced seizures ( J:127120 )
• mice are more sensitive to seizures induced with 85 mg per kg pentylenetetrazol than wild-type mice
absence seizures ( J:127120 )
• when stimulated with 5 and 8 Hz and high-amplitude (500 to 1100 uV) spike-wave discharge (SWD) is observed up to 50 times an hour
• during SWD mice pause and display stereotypical nodding and twitching of the orofacial region with some mice exhibiting subtle head jerk or turn that are not observed in wild-type mice
• Background Sensitivity: fewer mice exhibit symptomatic spike-wave discharge (SWD) on a C57BL/6 background than on a DBA/2J background with shorter SWD episodes (1.20+/-0.16 ms compared to 2.53+/-0.21 ms on DBA/2J background)
abnormal inhibitory postsynaptic currents ( J:127120 )
• miniature inhibitory postsynaptic current amplitudes of cortical neurons are lower than in wild-type mice (58.4+/-2.2 pA compared to 67.5+/-2.9 pA in wild-type mice)
• however, spontaneous inhibitory postsynaptic currents in the thalamic reticular nuclei are normal

behavior/neurological
increased susceptibility to pharmacologically induced seizures ( J:127120 )
• mice are more sensitive to seizures induced with 85 mg per kg pentylenetetrazol than wild-type mice
absence seizures ( J:127120 )
• when stimulated with 5 and 8 Hz and high-amplitude (500 to 1100 uV) spike-wave discharge (SWD) is observed up to 50 times an hour
• during SWD mice pause and display stereotypical nodding and twitching of the orofacial region with some mice exhibiting subtle head jerk or turn that are not observed in wild-type mice
• Background Sensitivity: fewer mice exhibit symptomatic spike-wave discharge (SWD) on a C57BL/6 background than on a DBA/2J background with shorter SWD episodes (1.20+/-0.16 ms compared to 2.53+/-0.21 ms on DBA/2J background)




Genotype
MGI:3835048
ht7
Allelic
Composition		 Gabrg2tm1Geh/Gabrg2+
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gabrg2tm1Geh mutation (1 available); any Gabrg2 mutation (40 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
reproductive system
reproductive system phenotype ( J:53823 )
N
• female mice exhibit normal fertility
reduced male fertility ( J:53823 )
• some mice exhibit reduced fertility while others are sterile




Genotype
MGI:6740192
ht8
Allelic
Composition		 Gabrg2tm1.2Rmac/Gabrg2+
Genetic
Background		 involves: C57BL/6 * C57BL/6J * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gabrg2tm1.2Rmac mutation (0 available); any Gabrg2 mutation (40 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
mortality/aging
sudden unexpected death in epilepsy ( J:306976 )
• many pups die in their home cage prior to weaning; in the third postnatal week (especially from P15-P17), pups die at a much higher rate than wild-type mice (14% vs. 0.4%)
• dead mice have frequent generalized tonic-clonic seizures before death, indicating sudden unexplained death in epilepsy (premature sudden death)
• sudden death resolves mostly after P17

behavior/neurological
increased vertical activity ( J:306976 )
• mice show increased number of vertical counts in the open-field test
increased locomotor activity ( J:306976 )
• in a 60-minute open-field test, mice travel farther than wild-type mice indicating hyperactivity
• however, mice do not show anxiety, exhibit normal spatial learning and memory in the Barnes maze, and no abnormalities in social interaction
seizures ( J:306976 )
• mice exhibit multiple types of spontaneous seizures with variable severity; most common ones include atypical absence, typical absence, and myoclonic seizures
increased susceptibility to pharmacologically induced seizures ( J:306976 )
• mice exhibit reduced threshold for pentylenetetrazol (PTZ)-induced seizures; 30 mg/kg PTZ induces myoclonic and generalized tonic-clonic seizures compared to absence-like seizures in most wild-type mice, whereas a dose of 60-80 mg/kg PTZ is required to induce generalized tonic-clonic seizures or seizure-related death in wild-type mice
myoclonus ( J:306976 )
• myoclonic seizures are very subtle and infrequent in adults
tonic-clonic seizures ( J:306976 )
• generalized tonic-clonic seizures are not seen in adults but are seen in pre-weaned pups
febrile seizures ( J:306976 )
• mice exhibit age-dependent hyperthermic seizures
• 3.7% of P15-P17 mice, 44.4% of P20, and 90% of P30 or older mice exhibit myoclonic seizures when exposed to 42 degrees Celcius for 30 minutes (hyperthermia)
• 0% of P15-P17 mice and P20 mice, and 33% of P30 and older mice exhibit generalized tonic-clonic seizures when exposed to hyperthermia
absence seizures ( J:306976 )
• atypical absence seizures are associated with behavioral arrest that lasts from a few seconds to several minutes and have irregular ictal high-amplitude spike-wave discharges (3-5 Hz) that are not always synchronized with the onset of behavioral arrest
• typical absence seizures generally last for a few seconds and are associated with symmetrical high-amplitude spike-wave discharges with rhythmic 6-8 Hz on ictal EEG; onset is is accompanied by time-locked EEG activity and sudden behavioral arrest
• on average, mice have 56 atypical and 12 typical absence seizures per day

nervous system
seizures ( J:306976 )
• mice exhibit multiple types of spontaneous seizures with variable severity; most common ones include atypical absence, typical absence, and myoclonic seizures
increased susceptibility to pharmacologically induced seizures ( J:306976 )
• mice exhibit reduced threshold for pentylenetetrazol (PTZ)-induced seizures; 30 mg/kg PTZ induces myoclonic and generalized tonic-clonic seizures compared to absence-like seizures in most wild-type mice, whereas a dose of 60-80 mg/kg PTZ is required to induce generalized tonic-clonic seizures or seizure-related death in wild-type mice
myoclonus ( J:306976 )
• myoclonic seizures are very subtle and infrequent in adults
tonic-clonic seizures ( J:306976 )
• generalized tonic-clonic seizures are not seen in adults but are seen in pre-weaned pups
febrile seizures ( J:306976 )
• mice exhibit age-dependent hyperthermic seizures
• 3.7% of P15-P17 mice, 44.4% of P20, and 90% of P30 or older mice exhibit myoclonic seizures when exposed to 42 degrees Celcius for 30 minutes (hyperthermia)
• 0% of P15-P17 mice and P20 mice, and 33% of P30 and older mice exhibit generalized tonic-clonic seizures when exposed to hyperthermia
absence seizures ( J:306976 )
• atypical absence seizures are associated with behavioral arrest that lasts from a few seconds to several minutes and have irregular ictal high-amplitude spike-wave discharges (3-5 Hz) that are not always synchronized with the onset of behavioral arrest
• typical absence seizures generally last for a few seconds and are associated with symmetrical high-amplitude spike-wave discharges with rhythmic 6-8 Hz on ictal EEG; onset is is accompanied by time-locked EEG activity and sudden behavioral arrest
• on average, mice have 56 atypical and 12 typical absence seizures per day
abnormal nervous system electrophysiology ( J:306976 )
• mice exhibit prolonged and high amplitude thalamocortical oscillations recorded from thalamic ventrobasal nucleus neurons (long period rhythmic neuronal firing activity with different action potential amplitudes) compared to very short spontaneous network bursts in wild-type mice
decreased miniature inhibitory postsynaptic current amplitude ( J:306976 )
• mice exhibit reduced mIPSC amplitudes recorded from somatosensory cortex layer V/VI pyramidal neurons

muscle
myoclonus ( J:306976 )
• myoclonic seizures are very subtle and infrequent in adults

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
generalized epilepsy with febrile seizures plus DOID:0060170 J:306976




Genotype
MGI:5474679
cn9
Allelic
Composition		 Gabrg2tm2Spet/Gabrg2+
Tg(Camk2a-tTA)1Mmay/0
Tg(tetO-cre)LC1Bjd/0
Genetic
Background		 involves: 129S1/Sv * 129X1/SvJ * BALB/c * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gabrg2tm2Spet mutation (0 available); any Gabrg2 mutation (40 available)
Tg(Camk2a-tTA)1Mmay mutation (8 available)
Tg(tetO-cre)LC1Bjd mutation (2 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
behavior/neurological
increased susceptibility to pharmacologically induced seizures ( J:194736 )
• induced by pentylenetetrazil in mice treated with doxycycline treatment between conception and P21 or not treated with doxycycline at all
• induced by pentylenetetrazil compared with Gabrg2tm2Spet heterozygotes
• doxycycline treatment between conception and P21 increased the time to first clonic seizure compared with mice not treated with doxycycline
• however, no seizure kindling effect is observed

nervous system
increased susceptibility to pharmacologically induced seizures ( J:194736 )
• induced by pentylenetetrazil in mice treated with doxycycline treatment between conception and P21 or not treated with doxycycline at all
• induced by pentylenetetrazil compared with Gabrg2tm2Spet heterozygotes
• doxycycline treatment between conception and P21 increased the time to first clonic seizure compared with mice not treated with doxycycline
• however, no seizure kindling effect is observed




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Send questions and comments to User Support. 		last database update
03/18/2025
MGI 6.24 		The Jackson Laboratory