Analysis Tools
behavior/neurological
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• when challenged to walk on a narrow beam, mice exhibit more foot-slips than controls and took twice as long to cross the beam, suggesting modest motor impairment
• mice do not exhibit overt ataxia or gait abnormalities during normal behavior
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nervous system
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• radial glial endfeet are mispositioned and fibers are disorganized extending beyond the pia mater into the neuronal ectopia in the areas where the basement membrane is disrupted
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• mice exhibit over-migration of some neurons through the marginal zone due to defects in the pial basement membrane
• however, migration of most neurons is normal
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• at E18.5, the basement membrane in the cerebellum exhibits focal ruptures, disrupting migration of granule cells
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• at E12.8, Cajal-Retzuis cells are malpositioned beyond the pial surface at defective basement membrane sites
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• in the pons, the defects primarily involved loss of neurons in the pontine grey nuclei
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• ectopic neurons, from both deep and superficial layers, are observed in the frontoparietal cortex due to over-migration of neurons through the marginal zone
• lamination beneath ectopic neurons is disorganized
• at E13.5, the pial basement membrane exhibits discontinuity that becomes more prominent at E14.5 and E16.5
• however, assembly of the basement membrane is normal
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• fusion of adjacent lobules, disruption of normal layering, and ectopic clusters of neurons, spanned the entire mediolateral axis of the rostral cerebellum, including the vermis as well as cerebellar hemispheres
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• rostral part of the cerebellum, encompassing lobules IV, was malformed in all homozygotes
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cellular
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• radial glial endfeet are mispositioned and fibers are disorganized extending beyond the pia mater into the neuronal ectopia in the areas where the basement membrane is disrupted
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• mice exhibit over-migration of some neurons through the marginal zone due to defects in the pial basement membrane
• however, migration of most neurons is normal
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• at E18.5, the basement membrane in the cerebellum exhibits focal ruptures, disrupting migration of granule cells
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• at E18.5, the basement membrane in the cerebellum exhibits focal ruptures, disrupting migration of granule cells
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