Analysis Tools
mortality/aging
| N |
• mice survive to adulthood
|
craniofacial
|
• 85% of mice exhibit increased curvature of the nasion vs 14% of wild-type controls
|
broad snout
(
J:344365
)
|
• mice exhibit a significantly broader snout than wild-type controls, as measured by the distance between the most lateral 3rd row of vibrissae
• however, no significant difference is observed in head width
|
behavior/neurological
| N |
• mice do NOT exhibit spontaneous seizures at any age and show normal behavior in the grip strength test and the hanging test at 1 month of age
|
|
• in a contextual fear conditioning task, mice exhibit significantly less freezing than wild-type controls in contextual testing 24 h after conditioning
|
|
• mice exhibit significantly less freezing in cued conditioning during pre-tone and tone
|
|
• mice exhibit novel environment-induced hyperactivity (total distance traveled) in the open field test
|
|
• in the Barnes maze task, 12-wk-old mice perform worse than wild-type controls at finding the target hole across the acquisition phase
|
|
• in the open field test, mice spend significantly more time exploring the periphery while avoiding the anxiety-provoking center
|
|
• dams are not good mothers; litters derived from wild-type males bred with heterozygous females are nursed by wild-type foster females
|
nervous system
|
• at E16.5, the proportion of radial glial cells is significantly increased in the developing cortex
|
|
• at E16.5, proportions of interneurons are consistently reduced in the developing cortex
|
|
• at P0, significantly less callosal axons cross the midline and the number of midline astroglia cells is decreased
• decreased midline crossing of commissural axons is associated with an abnormal arrangement of GFAP+ guidepost glia cells and a widening of the interhemispheric fissure
|
|
• embryonic brains exhibit abnormal density of layer-specific neurons in the cortex
• at E16.5, the frequency of cells co-expressing Satb2 and Ctip2 is significantly higher than in wild-type controls
• at E18.5, the proportion of cells expressing Tbr1 in layer V (co-expressing Ctip2) is significantly higher than in wild-type controls
|
|
• embryonic brains exhibit reduced cortical thickness
|
|
• at E18, all GFAP+ glia, including the glial wedge (GW), indusium griseum glia (IGG) and midline zipper glia (MZG) subtypes, are generally reduced
|
|
• at E16.5, the proportion of progenitor-like cells in the subventricular zone is significantly increased in the developing cortex whereas the proportion of ganglionic eminence cells is reduced
|
growth/size/body
|
• 85% of mice exhibit increased curvature of the nasion vs 14% of wild-type controls
|
broad snout
(
J:344365
)
|
• mice exhibit a significantly broader snout than wild-type controls, as measured by the distance between the most lateral 3rd row of vibrissae
• however, no significant difference is observed in head width
|
cellular
|
• at E16.5, the proportion of radial glial cells is significantly increased in the developing cortex
|
|
• abnormal epigenetic transcriptional regulation of corticogenesis
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| GAND syndrome | DOID:0070048 |
OMIM:615074 |
J:344365 | |
