Mouse Genome Informatics
ot
    Arxtm2Kki/Y
involves: 129S/SvEv * C57BL/6J
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• mice die within 1 day of birth (J:152416)

nervous system
• GABAergic progenitor cells migration is delayed until E14.5 whereas migration in wild-type mice occurs at E12.5 (J:152416)
• only 29% of the Arx+ GABAergic cells that migrate in wild-type mice reach the cortical plate by P0 (J:152416)
• radial and tangential migration of GABAergic neurons is impaired compared to in wild-type mice (J:152416)
• 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 (J:152416)
• at P0, the Arx+ MAP2- ventricular and subventricular zones of the striatum are thicker than in wild-type mice (J:152416)
• 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 (J:152416)

cellular
• GABAergic progenitor cells migration is delayed until E14.5 whereas migration in wild-type mice occurs at E12.5 (J:152416)
• only 29% of the Arx+ GABAergic cells that migrate in wild-type mice reach the cortical plate by P0 (J:152416)
• radial and tangential migration of GABAergic neurons is impaired compared to in wild-type mice (J:152416)

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