mortality/aging
• 60% die by P35, although a few survive for over 12 months
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nervous system
• Background Sensitivity: exhibit spontaneous seizures that are more severe on a mixed 129S4/SvJaeSor and C57BL/6J background than on a coisogenic 129S4/SvJaeSor background
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• most regions of the CNS displays severe hypomyelination
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behavior/neurological
• Background Sensitivity: exhibit shaking; phenotype is more severe on a mixed 129S4/SvJaeSor and C57BL/6J background than on a coisogenic 129S4/SvJaeSor background
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• decrease in limb mobility
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• Background Sensitivity: exhibit spontaneous seizures that are more severe on a mixed 129S4/SvJaeSor and C57BL/6J background than on a coisogenic 129S4/SvJaeSor background
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skeleton
N |
• do not exhibit skeletal defects
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craniofacial
N |
• do not exhibit cleft palate
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