mortality/aging
• premature death several months after development of the thoracolumbar kyphosis
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behavior/neurological
• range of motion in both fore and hindlimbs is limited
|
• evident at 3 monthes of age
• tremor involving primarily the trunk musculature, occurring more than 10 times a min and lasting less than 5 seconds
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abnormal gait
(
J:84278
)
• abnormal gait characterized by a reduction in average stride length and wide splaying of the hindlegs resulting in an increase in stride width
• secondary to rigid thoracolumbar kyphosis
|
growth/size/body
• failure to thrive
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• from about 12 weeks of age, mutants show growth retardation
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skeleton
• from about 12 weeks of age, mutants develop a progressive rigid thoracolumbar kyphosis such that by 9-10 months of age and 1 year of age, 50% and 85% of mutants are affected, respectively
• females are affected earlier than males
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muscle
• reduction in muscle bulk
|