growth/size/body
• lower body weight in males at 5-6 weeks of age, and in females from 4-14 weeks compared to controls
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behavior/neurological
• mice are more frightened by handlers, and are more difficult to catch by the tail compared to wild-type littermates
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• mice are more easily startled by sound
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• tremors in fore and hindlimbs is observed on balance beam or upon suspension by tail; abrupt undulating movements along spine are seen when mice are suspended
• during ambulation, shaking motion of ears, tail and body are observed in contrast to control littermates
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• impaired ability to counterbalance body weight on 4 paws when placed perpendicular to beam
• mutants move more rapidly on beam, with tendency to fall, compared to wild-type
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• mutants perform better on rotarod apparatus at 4, 8, and 15 weeks of age
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• increased rearing and increased climbing behavior compared to controls is observed
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• increased movement in cages is seen in males at 30- and 55-minute time points and in females from 10 to 60 minutes, along with increased climbing behavior
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nervous system
• in cerebrum, more axons in mutants (24.1%) are in smallest range of myelin thickness than in wild-type (12.8%); more wild-type cerebral axons are in higher size ranges (8.5% with .16-.2 um thickness; 7.5% with >.21 um myelin thickness) compared to mutants (5% and 0%); mutant axons show hypomyelination
• in spinal cord, less axons (23.2%) in mutants have lower sheath measurements than in wild-type (34.4%); mutant axons predominate in higher (13.6% at .65-.94 um; 10.6% at >.94%) compared to wild-type (4.5% and 1.4%, respectively); mutant axons are hypermyelinated
• average myelin sheath thickness in spinal cord of female mutants (0.2 um) is increased vs wild-type (.12 um)
• in cerebrum and spinal cord of mutants, reduced periodicity and compaction of myelin is displayed
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