Analysis Tools
behavior/neurological
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• hind limb and 4-limb grip strength is reduced at 6 months and 1 year
• however, no difference in front limb grip strength is seen
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nervous system
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• vacuoles in adaxonal Schwann cells
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• mice show degenerating myelinated axons, vacuoles in adaxonal Schwann cells, demyelinated axons, remyelinated axons (axons with myelin sheaths that are inappropriately thin for the axonal diameter, or myelinated axons partially surrounded by cellular processes), clusters of regenerated axons, and macrophages with myelin debris
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• the latency of the sciatic F-wave is prolonged in 12-month-old mice
• however, the amplitude and distal latency of the sciatic motor compound muscle action potentials (CMAPs), as well as the amplitude of the caudal nerve response are not different
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• 3-month-old mice show slowing of the sciatic nerve motor conduction velocity
• 6-month-old mice exhibit slower sciatic and caudal nerve motor conduction velocity
• 12-month-old mice exhibit slower sciatic, but not caudal, nerve motor conduction velocity
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cellular
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• vacuoles in adaxonal Schwann cells
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Charcot-Marie-Tooth disease X-linked dominant 1 | DOID:0110209 |
OMIM:302800 |
J:350504 | |
