nervous system
• mice show normal myelination at P15, however axons of peripheral nerves are hypermyelinated by P90
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• mice show normal myelination at P15, however axons of peripheral nerves are hypermyelinated by P90
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• mice lack mature BCR positive B cells
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• mice lack mature BCR positive B cells
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• at P15, the myelin of peripheral axons is thinner but axons are hypermyelinated at P90
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• at P15 and P90, 36.7% and 17.8% of axons with diameters >1 um are nonmyelinated, respectively compared to 3.5% and 0.8% in controls
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• axons are hypermyelinated at P90
• however, mice show rescue of Schwann cell development and Schwan cells are able to myelinate
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• axons of peripheral nerves are hypermyelinated at P90
• however, mice show normal numbers of Schwann cells
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• onion bulb structures are seen indicating myelin degeneration and regeneration
• however, stacking of myelin sheaths in compact myelin is unaffected
• noncompact myelin structures are more abundant
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• myelin sheaths are thicker at P15 and P30 and myelin continues to grow radially such that the mean myelin area in peripheral nerves at P90 is increased 6-fold
• increase in myelin thickness is particularly pronounced in small-diameter fibers
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• increase in the diameter of sciatic nerves due to massive hypermyelination and reduced axonal packing
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• myelination begins prematurely and mice show continuous myelin growth
• increase in the number of myelinated fibers per field at P0
• myelin abnormalities are seen at P30-P90 but not at P15, indicating that they result from massive hypermylination
• treatment with the mTORC1 inhibitor everolimus dampens myelin growth in the nerves of mutants and completely rescues the decreased axonal packing
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• ratio of compound action potentials in myelinated A and nonmyelinated C fibers (A/C fiber waves) is reduced and there is a reduction in numbers of active mechanoreceptive units after P60 indicating that many myelinated axons are not conducting
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• D-hair mechanoreceptors show increased conduction velocities at P30, however no differences were seen after P60
• however, conduction velocities of other mechanosensory fibers are unchanged
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
N |
• constitutively active Map2k1(MEK) rescues thymocyte development restoring single positive thymocyte and splenic T cell populations
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
N |
• rarely observe aberrantly myelinated small axons in mutants and the ratio of nonmyelinated to myelinated axons is unchanged
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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
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last database update 11/12/2024 MGI 6.24 |
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