Analysis Tools
growth/size/body
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• mice are approximately 30% smaller than wild-type mice
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• reduced weight is seen at weaning and continues throughout life
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nervous system
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• sciatic nerves at 3 months show an increase in the proportion of bundled axons that have a diameter of more than 1 um, indicating delayed or arrested sorting of some large axons from bundles
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• mice exhibit fewer total myelinated axons in sciatic nerves
• sciatic nerves at 3 months show an increase in unmyelinated axons that are in 1:1 relationships with Schwann cells, although most axons are smaller than 1 um in diameter
• however, the number of axons per bundle does not differ
• a decrease in the percentage of bundles containing between 11 and 20 axons is seen in sciatic nerves
• bundled axons of sciatic nerves are more likely to be incompletely enwrapped by Schwann cell processes, as evidenced by direct contact of axons with basal laminae
• however, mice do not show loss of myelin outfoldings and show normal myelination, with no alterations in myelin thickness or diameter of myelinated axons
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reproductive system
behavior/neurological
| N |
• overt motor or gait deficiencies are not seen at P38 or 17 months of age
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Charcot-Marie-Tooth disease type 4B3 | DOID:0110194 |
OMIM:615284 |
J:326685 | |
