behavior/neurological
• a test in which an auditory cue was coupled with a foot shock failed to elicit a startle response to the auditory cue after training in mutant mice
|
• the amplitude of the acoustic startle response was larger in mutant mice
|
limb grasping
(
J:38887
)
• mice often hold their tails off the surface when walking and sometimes arch the tail over their backs
|
• females take poor care of offspring
|
growth/size/body
• mice weigh approximately 25% less than littermate controls before weaning, mice weigh approximately 50% less than littermate controls throughout adulthood
|
hearing/vestibular/ear
N |
• mice exhibit normal auditory brainstem (ABR) responses indicating that mutant mice can hear normally
|
• elevated threshold
|
hematopoietic system
• slightly higher than controls, but within values expected for the background strain
|
reproductive system
• males rarely breed
|
nervous system
N |
• EEG patterns were similar to controls
• normal histopathology of the cerebrum, thalamus, brainstem, spinal cord was noted
|
• most lobules are shortened in length
(J:70222)
• lobulation began between P2 and P4 instead of P0 as in controls
(J:74540)
• lobulation continued to be delayed past the first postnatal week
(J:74540)
|
• folia is reduced to 7 as contrasted with the wild-type folia count of 10; foliation pattern is also perturbed
(J:38887)
• absence of intraculmenate fissure that divides the culmen into two lobules, 4 and 5
(J:38887)
• absence of the declivis 6a and 6b
(J:38887)
• absence of fissura seconda that separates the pyramis 8 from the uvula 9
(J:38887)
• absence of uvula 9b and 9c
(J:38887)
• the precentral fissure between lobules II and III is rudimentary
(J:70222)
• at P0 and P2, cerebella lacked fissures unlike controls
(J:74540)
• the prepyramidal fissure appeared at P8
(J:74540)
|
• in the pyramidal cell layer, pyramidal cells were less densely packed compared to wild-type mice, and occasionally appears as isolated cells
|
• pyramidal cells of the CA1, CA2, and CA3 region were scattered into the external and deep plexiform layers of the hippocampus
|
• Purkinje cells were observed in the granule cell layer and in the white matter
(J:38887)
• a failure of Purkinje cell migration during development results in widespread ectopia in the adult
(J:70222)
• the cells are not arranged in the normal monolayer in the cerebellum and appear in the deep core of the cerebellum, the granular layer, and the intralobular white matter
(J:70222)
• total numbers of Purkinje cells appeared similar in mutant mice and controls
(J:70222)
• ectopic Purkinje cells were those primarily not expressing zebrin II
(J:70222)
• at P0, Purkinje cells are scattered throughout the white matter
(J:74540)
• at P4, some Purkinje cells had aligned in a single layer, but many remained at ectopic sites
(J:74540)
|
• reduced number of granule cells were observed at 5 months of age
|
• mineral deposits seen in the ventral vermis of the midline
(J:38887)
• vermis is reduced in rostro-caudal length by more than one third
(J:70222)
|
• reduced in overall size compared to controls
|
• midsagittal area of the mutant cerebellum is ~60% that of control mice
|
• a weaker PPI was seen compared to controls
|