craniofacial
• the skull is shorter and misshapen with incomplete ossification and some have an open foramen
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small ears
(
J:223062
)
• ear pinnae are less than half the normal size
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skeleton
• the skull is shorter and misshapen with incomplete ossification and some have an open foramen
|
• a significant reduction in bone mineral content of the skull is found in males, with a trend toward significance in females
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• the skull shows incomplete ossification
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nervous system
• some neurons in the dorsal root ganglia and trigeminal ganglion were found to be apoptotic or necrotic at 3 weeks of age
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muscle
• ascending dystonia beginning at 2 weeks of age and progressing until death at approximately 3 weeks of age
• elongated step/push gait with the hind limbs, and impaired grasping reflex including gnarled wrists are found at 3 weeks of age, and the kinked tail appears to have a neurological cause
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behavior/neurological
• ascending dystonia beginning at 2 weeks of age and progressing until death at approximately 3 weeks of age
• elongated step/push gait with the hind limbs, and impaired grasping reflex including gnarled wrists are found at 3 weeks of age, and the kinked tail appears to have a neurological cause
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mortality/aging
• most homozygotes die by 3 weeks of age and none have survived to breeding age
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limbs/digits/tail
kinked tail
(
J:223062
)
• the bone structure of the kinked tail is normal and the tail straightens significantly after death indicating a neurological cause of the kinked tail
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growth/size/body
small ears
(
J:223062
)
• ear pinnae are less than half the normal size
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• very runted body size compared with littermate control
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hearing/vestibular/ear
small ears
(
J:223062
)
• ear pinnae are less than half the normal size
|