nervous system
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• hypomyelination with swellings of the inner tongue of myelin
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Allele Symbol Allele Name Allele ID |
Magtm1Mtg targeted mutation 1, Dirk Montag MGI:2384092 |
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Summary |
5 genotypes
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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• hypomyelination with swellings of the inner tongue of myelin
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
N |
• despite abnormalities in myelin, no defects in nerve conduction are detected
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• unlike in wild-type controls, very few axons have periaxonal cytoplasmic collars that extend more than half the circumference of the myelin sheath
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• at 2 - 4 months of age in the optic nerve, oligodendrocytes contain regions with cytoplasm within the compact myelin that upon closer examination reveal more than one concentric myelin sheath associated with an axon
• some axons are associated with up to 4 individual myelin sheaths
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• at P10 and P11 in the retinal ganglion, fewer myelinated axons are seen suggesting a delay in myelination
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• at 8 and 9 months of age in the spinal cord, oligodendrocyte processes are enlarged and contain myelin like vesicles and multivesiculated bodies
• in severely enlarged processes amorphous and granular osmiophilic material appeared side by side with vesicular inclusions or mitochondria
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• at 8 months of age, Schwann cells with contorted myelin sheaths or myelin debris and Schwann cells not in contact with axons are seen
• at 8 months of age, Schwann cells occasionally contain degenerating myelin and axonal debris
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• at 8 and 9 months of age in the spinal cord, scattered thickened myelinated fibers with moderately osmiophillic material between the myelin sheath and the axon are seen
(J:38760)
• at 26 weeks of age, onion bulb like structures are seen in the femoral quadriceps nerve
(J:78646)
• at 8 months of age, onion bulbs consisting of myelinating Schwann cells and concentrically arranged Schwann cell processes each covered by a basal lamina are seen
(J:106609)
• decreased diameter of sciatic nerve axons
(J:296495)
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• at 26 weeks of age, degenerating axons and degenerating myelin are seen in the femoral quadriceps nerve
(J:78646)
• however, at 26 weeks of age very few degenerating fibers are seen in the cutaneous (saphenous) nerve branch
(J:78646)
• at 8 months of age, many axons show signs of degeneration
(J:106609)
• degenerating axons have small diameters and high electron density or axoplasm containing numerous small vesicles and/or cellular debris
(J:106609)
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• presence of onion bulb structures indicates demyelination induced Schwann cell proliferation
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
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♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• at 4 weeks of age, onion bulb like structures are seen in the pectineus and quadriceps nerves
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• at 8 weeks of age in single fiber preparations many fibers show signs of myelin degeneration
• degeneration is preferentially associated with the paranodal regions of the fibers
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• at 4 weeks of age, degenerating axons and degenerating myelin are seen in the femoral quadriceps nerve
• at 8 weeks of age the number of abnormal axons is increased compared to mice at 4 weeks of age
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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 12/17/2024 MGI 6.24 |
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