Analysis Tools
nervous system
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• at 6 months of age, optic nerves show no alterations in myelin sheath thickness, percentage of myelinated axons, or frequency of degenerating/degenerated axons relative to those in controls mice
• no axonopathy, astrogliosis or microgliosis is observed
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• at P75, mice exhibit numerous myelin outfoldings in the CNS (e.g. optic nerve)
• myelin outfoldings do not exhibit a pin-needle-like shape but represent large sheets of compacted multilayered membrane stacks that extend considerably away from the myelinated axon
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• mice display disrupted assembly of septin filaments in CNS myelin
• abundance of all myelin septins (SEPT2, SEPT4, SEPT7,SEPT8) is strongly reduced in myelin purified from mutant brains
• abundance of the membrane phospholipid phosphatidylinositol (4,5)-bisphosphate (PtdIns(4,5)P2) is reduced in myelin purified from mutant brains
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• at 6 months of age, nerve conduction velocity in the spinal cord is reduced by 15.5%
• however, no nodal or paranodal abnormalities are observed
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