All Phenotypes Lhx6<tm2Vpa> MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Lhx6^tm2Vpa/Lhx6^tm2Vpa	involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6	MGI:3703334
hm2	Lhx6^tm2Vpa/Lhx6^tm2Vpa	involves: 129P2/OlaHsd * C57BL/6	MGI:5498873
ht3	Lhx6^tm1Vpa/Lhx6^tm2Vpa	involves: 129P2/OlaHsd * C57BL/6	MGI:5498872
cn4	Gt(ROSA)26Sor^tm1(EYFP)Cos/Gt(ROSA)26Sor⁺ Lhx6^tm2Vpa/Lhx6^tm2Vpa Tg(Nkx2-1-cre)1Wdr/0	involves: 129P2/OlaHsd * 129X1/SvJ	MGI:5491493
cx5	Gad1^tm1Tama/? Lhx6^tm2Vpa/Lhx6^tm2Vpa	involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6 * CBA	MGI:3703343

Allelic Composition	Lhx6^tm2Vpa/Lhx6^tm2Vpa
Genetic Background	involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lhx6^tm2Vpa mutation (0 available); any Lhx6 mutation (27 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

postnatal lethality, complete penetrance ( J:119476 )

• fail to thrive and die within 2 weeks of birth

nervous system

abnormal neuronal migration ( J:119476 )

• at E13.5, the front of tangentially migrating GAD67+ cells in the cortex is located in a more ventrolateral position compared to heterozygous controls

• at E15.5,very few GAD67+ cells are found in the marginal zone or the cortical plate, unlike in heterozygous controls

decreased neuronal migration ( J:119476 )

• reduced migration of cells away from medial ganglionic eminence explants from E13.5 embryos

abnormal brain interneuron morphology ( J:119476 )

• altered distribution of GAD67+ cells in the neocortex and hippocampus

• however, the number of GAD67+ cells is normal in the neocortex and hippocampus

• reduced number of PV+ and Sst+ interneurons in the neocortex and hippocampus

• Cr+ interneurons are reduced in the neocortex but unchanged in the hippocampus

abnormal hippocampus morphology ( J:119476 )

• interneurons are found mostly in the deeper layers (stratum radiatum and stratum lacunosum moleculare) and not in the stratum oriens or pyramidal cell layer

• however, no reduction in the total number of interneurons is detected

abnormal cerebral cortex morphology ( J:119476 )

• interneurons accumulate in the upper and deeper layers, rather than having a relatively uniform distribution across the layers as in control mice

• however, no reduction in the total number of interneurons is detected

behavior/neurological

weakness ( J:119476 )

• develop general weakness within 2 weeks of birth

cellular

abnormal neuronal migration ( J:119476 )

• at E13.5, the front of tangentially migrating GAD67+ cells in the cortex is located in a more ventrolateral position compared to heterozygous controls

• at E15.5,very few GAD67+ cells are found in the marginal zone or the cortical plate, unlike in heterozygous controls

decreased neuronal migration ( J:119476 )

• reduced migration of cells away from medial ganglionic eminence explants from E13.5 embryos

Allelic Composition	Lhx6^tm2Vpa/Lhx6^tm2Vpa
Genetic Background	involves: 129P2/OlaHsd * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lhx6^tm2Vpa mutation (0 available); any Lhx6 mutation (27 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

abnormal brain interneuron morphology ( J:198547 )

• reduced Pva+ cortical interneurons

decreased neuron number ( J:198547 )

• reduced Pva+ cortical interneurons

abnormal inhibitory postsynaptic currents ( J:198547 )

• decreased frequency

• however, amplitude is normal

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

nervous system phenotype ( J:198547 )

N	• mice exhibit normal MGE-derived cortical interneurons and spontaneous inhibitory postsynaptic currents

increased susceptibility to pharmacologically induced seizures ( J:198547 )

• in kainic acid treated mice

environmentally induced seizures ( J:198547 )

• under mild stress

tonic-clonic seizures ( J:198547 )

abnormal spike wave discharge ( J:198547 )

• interictal spikes in all mice

abnormal neuron differentiation ( J:198547 )

• after 4 weeks of age, mice exhibit histopathological evidence of seizures (NPY, EdU and Cr staining) with evidence of enhanced neurogenesis unlike Lhx6^tm1Vpa heterozygotes

abnormal brain interneuron morphology ( J:198547 )

• mice exhibit reduced Pva+ interneurons in the dentate gyrus and widespread loss of Sst+ interneurons in the cortex (preceding onset of tonic-clonic seizures) compared with Lhx6^tm1Vpa heterozygotes

• however, mice exhibit normal numbers of Pva+ interneurons is in the somatosensory cortex and the CA1 region of the hippocampus and a normal complement of MGE-derived cortical interneurons

decreased neuron number ( J:198547 )

• however, mice exhibit normal numbers of Pva+ interneurons is in the somatosensory cortex and the CA1 region of the hippocampus and a normal complement of MGE-derived cortical interneurons

increased miniature inhibitory postsynaptic current frequency ( J:198547 )

• 6-fold higher frequency of dendritic mIPSCs compared with Lhx6^tm1Vpa heterozygotes

behavior/neurological

increased susceptibility to pharmacologically induced seizures ( J:198547 )

• in kainic acid treated mice

environmentally induced seizures ( J:198547 )

• under mild stress

tonic-clonic seizures ( J:198547 )

abnormal spike wave discharge ( J:198547 )

• interictal spikes in all mice

cellular

abnormal neuron differentiation ( J:198547 )

• after 4 weeks of age, mice exhibit histopathological evidence of seizures (NPY, EdU and Cr staining) with evidence of enhanced neurogenesis unlike Lhx6^tm1Vpa heterozygotes

Allelic Composition	Gt(ROSA)26Sor^tm1(EYFP)Cos/Gt(ROSA)26Sor⁺ Lhx6^tm2Vpa/Lhx6^tm2Vpa Tg(Nkx2-1-cre)1Wdr/0
Genetic Background	involves: 129P2/OlaHsd * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^tm1(EYFP)Cos mutation (11 available); any Gt(ROSA)26Sor mutation (993 available)
Lhx6^tm2Vpa mutation (0 available); any Lhx6 mutation (27 available)
Tg(Nkx2-1-cre)1Wdr mutation (0 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

nervous system

abnormal brain interneuron morphology ( J:196342 )

• mice exhibit impaired somatostatin+ cortical interneuron differentiation compared with control mice

Allelic Composition	Gad1^tm1Tama/? Lhx6^tm2Vpa/Lhx6^tm2Vpa
Genetic Background	involves: 129P2/OlaHsd * 129S4/SvJae * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gad1^tm1Tama mutation (2 available); any Gad1 mutation (65 available)
Lhx6^tm2Vpa mutation (0 available); any Lhx6 mutation (27 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

mortality/aging

lethality throughout fetal growth and development, incomplete penetrance ( J:119476 )

• most die around E15.5

nervous system

abnormal neuronal migration ( J:119476 )

• at E13.5, migration of cortical interneurons is delayed and cells are not all allocated to the appropriate migratory streams

cellular

abnormal neuronal migration ( J:119476 )

• at E13.5, migration of cortical interneurons is delayed and cells are not all allocated to the appropriate migratory streams

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