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Phenotypes Associated with This Genotype
Genotype
MGI:4359205
Allelic
Composition
Arxtm2Kki/Y
Genetic
Background
involves: 129S/SvEv * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Arxtm2Kki mutation (2 available); any Arx mutation (19 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mice die within 1 day of birth

nervous system
• GABAergic progenitor cells migration is delayed until E14.5 whereas migration in wild-type mice occurs at E12.5
• only 29% of the Arx+ GABAergic cells that migrate in wild-type mice reach the cortical plate by P0
• radial and tangential migration of GABAergic neurons is impaired compared to in wild-type mice
• unlike in wild-type mice, SST+ GABAergic interneurons accumulate in the subventricular zone of the ventral striatum (around the nucleus accumbens) whereas no SST+ cells are detected in the mantle zone of the striatum
• at P0, the Arx+ MAP2- ventricular and subventricular zones of the striatum are thicker than in wild-type mice
• unlike in wild-type mice, SST+ GABAergic interneurons accumulate in the subventricular zone of the ventral striatum (around the nucleus accumbens) whereas no SST+ cells are detected in the mantle zone of the striatum

cellular
• GABAergic progenitor cells migration is delayed until E14.5 whereas migration in wild-type mice occurs at E12.5
• only 29% of the Arx+ GABAergic cells that migrate in wild-type mice reach the cortical plate by P0
• radial and tangential migration of GABAergic neurons is impaired compared to in wild-type mice

Mouse Models of Human Disease
OMIM ID Ref(s)
Lissencephaly, X-Linked, 2; LISX2 300215 J:197588


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
09/20/2016
MGI 6.05
The Jackson Laboratory