Phenotypes Associated with This Genotype

Genotype
MGI:5498872

• Allelic Composition: Lhx6^tm1Vpa/Lhx6^tm2Vpa
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6

• ♀: 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 )

• 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

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