nervous system
• infrequently fibers may contain both infolds and outfolds
• myelin infolds and outfolds are seen on nerve fibers of the sciatic and saphenous nerves
• outfolds are seen on both small and large caliber fibers and consist of one or more satellite loops of myelin that sometimes contain axoplasm-like material
• infolds consist of myelin folded into the axon and may dramatically alter the profile of the axon
• the percentage of fibers with infolds or outfolds increases with age as does the complexity of the folds
• folds are most prominent adjacent to nodes of Ranvier which alter the shape of the node but do not affect its overall organization
• however, myelin thickness is not decreased, degenerating axons are only rarely seen and onion bulb formations seen in human patients with CMT4B are not seen
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• decreased motor nerve conduction velocity at 8 months of age
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behavior/neurological
• some older mice display wide placement of the hind limbs when placed on a flat surface
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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:133391 |