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Allele Symbol Allele Name Allele ID |
Tg(Mpz)88.4Mfel transgene insertion 88.4, M Laura Feltri MGI:2384594 |
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| Summary |
3 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• myelin sheaths are thinner
• myelin lamellae are widened
• however, mice show improved hypomyelination
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• abnormal compaction is not improved
• two types of abnormally compacted fibers are seen: rare fibers in which both the intraperiod and major dense line are absent and majority of fibers with myelin that appears compacted but has abnormal periodicity due to a wide (but not absent) intraperiod line
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• myelin sheaths are thinner
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• tomacular are readily seen at P28
• tomacula are not improved and are worsened
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• hypomyelination is only slightly improved
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice exhibit variable signs of muscle weakness such as clenching of the apws, dragging of hind limbs and floppy tail
• symptoms are least severe in this line
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• sciatic nerves show uncompaction of the myelin sheath
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• sciatic nerves show hypomyelination
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Charcot-Marie-Tooth disease type 1B | DOID:0110152 |
OMIM:118200 |
J:77658 | |
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 06/12/2024 MGI 6.13 |
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