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Phenotypes Associated with This Genotype
Genotype
MGI:5825304
Allelic
Composition
Wdfy3tm1a(KOMP)Mbp/Wdfy3tm1a(KOMP)Mbp
Genetic
Background
C57BL/6N-Wdfy3tm1a(KOMP)Mbp
Cell Lines DEPD00523_5_G11
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wdfy3tm1a(KOMP)Mbp mutation (0 available); any Wdfy3 mutation (65 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Wdfy3tm1a(KOMP)Mbp/Wdfy3tm1a(KOMP)Mbp mice exhibit severe neurodevelopmental defects

mortality/aging
• homozygotes die perinatally for unknown reasons

nervous system
• analysis of cortical layer-specific markers Tbr1 (layer VI) and Ctip2 (layer V) revealed focal anomalies in the somatosensory and motor cortices at P0
• newborn pups exhibit more severe neurodevelopmental defects than Wdfy3disc homozygotes
• ~13% increase in overall cerebral size relative to wild-type controls
• at E18.5, mice exhibit widespread focal cortical dysplasias that disrupt most cortical layers and invade the marginal zone
• focal cortical dysplasias occur at a higher frequency than in Wdfy3disc homozygotes
• newborn brains show an even more drastic thinning and lengthening of the neocortex relative to that in Wdfy3disc homozygotes
• abnormal cortical layer formation through intermingling of Ctip2+ neurons with Tbr1+ neurons in layer VI
• 16% reduction in neocortical thickness only in the most lateral position, with a trend towards thinning throughout the cortex
• heterotopias are found in the somatosensory cortex and more dorsally in the motor area and can affect the hippocampus, unlike in Wdfy3disc homozygotes where they are restricted to lateral aspects of the somatosensory area of the neocortex

cellular
• analysis of cortical layer-specific markers Tbr1 (layer VI) and Ctip2 (layer V) revealed focal anomalies in the somatosensory and motor cortices at P0


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
02/12/2019
MGI 6.13
The Jackson Laboratory