Analysis Tools
growth/size/body
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• mice exhibit lower body weight, especially males
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behavior/neurological
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• signs of motor deficits are seen as early as 4 weeks of age and are exacerbated with age
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• mice exhibit decreased latency to fall from the rotarod beginning at 4 weeks of age
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• mice exhibit gait abnormalities, especially in the stride length and toe spreading parameters
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• when lifted by the tail, mice cannot spread their hind limbs, suggesting limb muscle weakness
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nervous system
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• although a shift in the axonal size distribution is seen, the hypomyelination does not lead to apparent axonal loss
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• mice begin to show thinning of the myelin sheath at 4 weeks of age
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• progressive sciatic nerve degeneration
• occasional onion bulb, axons without myelination, excess collagen deposition, and infiltration of macrophages are seen
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• degeneration of myelin sheaths begins at 4 weeks of age and is severe at 12 weeks of age
• older mice exhibit severe demyelination of the sciatic nerves, indicating progressive nerve degeneration
• G ratio of nerve fibers is increased, suggesting demyelinating neuropathy
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• amplitudes of compound muscle action potentials are reduced in the sciatic nerve of 8-week-old mice
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• sciatic nerve shows decreased motor nerve conduction velocity in 8-week-old mice
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neoplasm
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Charcot-Marie-Tooth disease type 4D | DOID:0110186 |
OMIM:601455 |
J:330126 | |
