Analysis Tools
behavior/neurological
|
• 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
|
nervous system
|
• 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
|
|
• at E18.5, the basement membrane in the cerebellum exhibits focal ruptures, disrupting migration of granule cells
|
|
• 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
|
|
• at E12.8, Cajal-Retzuis cells are malpositioned beyond the pial surface at defective basement membrane sites
|
|
• in the pons, the defects primarily involved loss of neurons in the pontine grey nuclei
|
|
• 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
|
|
• 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
|
|
• rostral part of the cerebellum, encompassing lobules IV, was malformed in all homozygotes
|
cellular
|
• 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
|
|
• at E18.5, the basement membrane in the cerebellum exhibits focal ruptures, disrupting migration of granule cells
|
|
• 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
|
|
• at E18.5, the basement membrane in the cerebellum exhibits focal ruptures, disrupting migration of granule cells
|
