Allele Symbol Allele Name Allele ID |
Lamc1tm1Strl targeted mutation 1, Sidney Strickland MGI:2683482 |
||||||||||||||||||||
Summary |
4 genotypes
|
|
|
♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
|
|
♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• with scattered small myotubues and central nuclei in the absence of obvious endomysial fibrosis but an increase in interstitial collagen fibrils
|
• in the quadriceps
|
• in the lower back and hindlimbs
|
• smaller muscle diameter
|
• 7-fold increase in Schwann cells that do not myelinate
|
• areas of amyelination with 2-fold reduction in myelinated axons and near-complete absence of enveloped axons and naked axons
|
• slightly tremulous and retracted inward limbs when suspended by tail
|
• with difficulty in ambulation
|
• 2-fold decrease of Schwann cell lamellipodial processesed basement membrane
|
• with scattered small myotubues and central nuclei in the absence of obvious endomysial fibrosis but an increase in interstitial collagen fibrils
|
|
|
♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• in the most severely affected mutants, motor defects are also seen in the front legs
|
• mutants appear normal for 2-3 weeks after birth but by 4 weeks, begin to show hind leg weakness that leads to completely paralyzed hind legs by 3 months of age
|
• Schwann cells are present in nerves but do not differentiate; they have a discontinuous basal lamina, are arrested at the premyelination stage and do not form myelin
|
• defect in axon sorting and myelination of the dorsal root of the spinal cord
• sciatic nerves are only partially myelinated and have large bundles of naked, unsorted axons
|
• defect in axon sorting and myelination of the dorsal root of the spinal cord
|
• sciatic nerves are only partially myelinated and have large bundles of naked, unsorted axons
|
• defect in axon sorting and myelination of the dorsal root of the spinal cord; more severe than in dorsal root
|
• Schwann cells in nerves have normal proliferation and apoptosis during development however, they fail to sort and myelinate axons
|
• regeneration of the sciatic nerve after injury is impaired
|
• regeneration of the sciatic nerve after injury is impaired
|
• muscular atrophy is seen by 3 months of age
|
|
|
♀ | phenotype observed in females |
♂ | phenotype observed in males |
N | normal phenotype |
• in mutant nerves, Schwann cells populate the nerve but fail to sort and myelinate axons
|
• mutant sciatic nerves are undermyelinated even though many Schwann cells are present
|
• in mutant nerves, Schwann cells populate the nerve but fail to sort and myelinate axons
|
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO) |
||
Citing These Resources Funding Information Warranty Disclaimer, Privacy Notice, Licensing, & Copyright Send questions and comments to User Support. |
last database update 07/05/2024 MGI 6.24 |
![]() |
|