Phenotypes Associated with This Genotype

Genotype
MGI:5810136

• Allelic Composition: \Bicd2^tm1Hgrd/\Bicd2^tm1Hgrd; \Tg(GFAP-cre)25Mes/0
• Genetic Background: involves: C57BL/6 * FVB/N

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

Disrupted laminar organization of the cortex in Bicd2^tm1Hgrd/Bicd2^tm1Hgrd Tg(GFAP-cre)25Mes/0 mice

mortality/aging

premature death ( J:210236 )

• mice with hydrocephalus die by 3-4 weeks of age

• mice without hydrocephalus survive to >8 weeks

nervous system

abnormal neuronal migration ( J:210236 )

• FoxP2, a marker normally present in deep corticofugal layer V and VI pyramidal neurons, is abnormally distributed in superficial lamina, consistent with impaired radial migration

• corticofugal axonal trajectories (labelled with anti-neurofilament M antibody) arise in superficial instead of deep cortical layers

abnormal cerebellar granule cell migration ( J:210236 )

• mice exhibit the same cerebellar granule cell migration defects observed in Bicd2^tm1.1Hgrd homozygotes

hydrocephaly ( J:210236 )

• some mice develop less severe hydrocephalus than that observed in Bicd2^tm1.1Hgrd mice

• others do not develop hydrocephalus

decreased corpus callosum size ( J:210236 )

• mice exhibit a thin corpus callosum

decreased brain external capsule size ( J:210236 )

• mice exhibit a thin capsula externa

abnormal hippocampus layer morphology ( J:210236 )

• mice without hydrocephalus exhibit disrupted laminar organization in the hippocampus

abnormal hippocampus pyramidal cell layer ( J:210236 )

• at P20, the hippocampus pyramidal cell layer appears disorganized in the CA1 region

abnormal stratification in cerebral cortex ( J:210236 )

• mice without hydrocephalus exhibit disrupted laminar organization in the cerebral cortex

• FoxP2, a marker normally present in deep corticofugal layer V and VI pyramidal neurons, is abnormally distributed in superficial lamina

• laminar organization of NeuN+ cells is absent

abnormal cerebellar layer morphology ( J:210236 )

• mice without hydrocephalus exhibit disrupted laminar organization in the cerebellar cortex

absent cerebellar granule layer ( J:210236 )

• mice do not develop an internal granule cell layer

cellular

abnormal neuronal migration ( J:210236 )

• FoxP2, a marker normally present in deep corticofugal layer V and VI pyramidal neurons, is abnormally distributed in superficial lamina, consistent with impaired radial migration

• corticofugal axonal trajectories (labelled with anti-neurofilament M antibody) arise in superficial instead of deep cortical layers

abnormal cerebellar granule cell migration ( J:210236 )

• mice exhibit the same cerebellar granule cell migration defects observed in Bicd2^tm1.1Hgrd homozygotes