Analysis Tools
behavior/neurological
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• mild limb clamping seen starting at 2 months of age
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• impaired performance on a rotarod at 12 months but not at 4 months of age
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nervous system
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• some sciatic nerve fibers without myelin misfoldings show widening of the periaxonal space
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• infoldings and outfoldings of the entire myelin sheath of sciatic nerve fibers are seen by 3 weeks of age and become progressively more numerous and complex with age
• in ventral and dorsal roots misfoldings are seen faintly at 4 months of age and more obviously at 15 months of age
• infoldings present as finger-like inversions of the sheath or circular inclusions within the nerve fiber that may entrain Schwann cell cytoplasm and displace axoplasm
• complex misfoldings are preferentially found in nodal and paranodal regions
• no gross loss of myelinated axons, Schwann cell onion bulb formations, or abnormalities in myelin compaction or periodicity are detected
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• degeneration of whole Scwann cell-axon units in the sciatic nerve are seen sporadically at 4 months and more frequently at 15 months of age
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• reduced compound muscle action potential amplitude in the sciatic nerve at 12 months but not at 4 months of age
• F-wave latency is slightly but not significantly increased
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reproductive system
| N |
• unlike mice null for Mtmr2, no signs of impaired spermatogenesis are detected
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Charcot-Marie-Tooth disease type 4B2 | DOID:0110190 |
OMIM:604563 |
J:133042 | |
