Analysis Tools
mortality/aging
|
• mice die between P18 and P21
|
nervous system
|
• at P14, Schwann cell nuclei are irregularly shaped
• Schwann cells do not progress beyond the promyelinating phase and continue to proliferate unlike wild-type cells
|
|
• at P18, mice exhibit extensive sprouting at the neuromuscular junctions unlike in wild-type mice
• however, no abnormal sprouting is observed at P14
|
|
• in 39% of mice
|
|
• the nodal structure of the sciatic nerve is abnormal
|
|
• at P14, the number of myelinating profiles is decreased, large caliber axons have no myelin sheaths, and myelin sheaths observed are thinner than in wild-type mice
• however, there is no evidence of demyelination or remyelination
|
|
• between P18 and P21 with evidence of conduction block
|
behavior/neurological
|
• mice exhibit weakness in the hindlimbs at P14 and weakness in the forelimbs at P18 unlike wild-type mice
|
|
• at P14, mice exhibit slowness and gait abnormality unlike wild-type mice
|
|
• within 4 to 7 days of P14 mice exhibit weakness leading to hindlimb paralysis unlike wild-type mice
|
growth/size/body
weight loss
(
J:145949
)
|
• after P14
|
respiratory system
|
• at P18
|
integument
| N |
• skin histology is normal
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Charcot-Marie-Tooth disease type 4E | DOID:0110195 |
OMIM:605253 |
J:145949 | |
