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Allele Symbol Allele Name Allele ID |
Plectm4Gwi targeted mutation 4, Gerhard Wiche MGI:3721885 |
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| Summary |
6 genotypes
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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 |
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• following treatment with tamoxifen and tape stripping (10 times), mice exhibit increased transepidermal water loss not observed in control Plec1tm4Gwi homozygotes
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• following treatment with tamoxifen and tape stripping (10 times), mice exhibit increased transepidermal water loss not observed in control Plec1tm4Gwi homozygotes
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• following treatment with tamoxifen and tape stripping (10 times), mice develop small blisters
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• skin around lesions induced by treatment with tamoxifen and tape stripping (10 times) is more fragile
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• decreased survival rates starting at 6 months of age
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| N |
• no abnormal muscle phenotype in early months of life
• extensor digitorum longus is normal at 16 months of age
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• focal disorganization of contractile apparatus
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• mitochondria lose association with Z-discs of muscle fibers
• mitochondria found in focal aggregates in sarcoplasm and subsarcolemmal regions
• numbers of mitochondria in muscle fibers is reduced
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• soleus muscle appears pale at 8 weeks of age and pathological changes are seen at 6 months
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• detached from the contractile apparatus in the soleus and the diaphragm
• 61% of fibers with focal detachments at 8 weeks
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• inner structure of fibers is disorganized
• eosinophylic inclusions are present under the sarcolemma
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• numerous necrotic fibers are found in the soleus at 8 weeks
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• hypertrophic and split fibers in both the soleus and diaphragm in older mice
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• centrally nucleated fibers are present in the soleus at 8 weeks
• at 3 months, 35% of fibers in exercised mice are centrally nucleated (45% at 12 months)
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• slight reduction in total number of fibers
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• loss of muscle mass observed in some mice at 16 months of age
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• atrophic fibers found in then soleus at 1 year
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| N |
• no heart pathologies at 12 months
• no dilated or hypertrophic cardiomyopathies at 16 months of age
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• increased connective tissue at 16 months of age
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• focal disorganization of contractile apparatus
|
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• decreased endurance during voluntary wheel running
|
|
• mitochondria lose association with Z-discs of muscle fibers
• mitochondria found in focal aggregates in sarcoplasm and subsarcolemmal regions
• numbers of mitochondria in muscle fibers is reduced
|
|
• numerous necrotic fibers are found in the soleus at 8 weeks
|
|
• decreased endurance during voluntary wheel running
|
|
• soleus muscle appears pale at 8 weeks of age and pathological changes are seen at 6 months
|
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• mice die 1 to 3 days after birth of malnutrition likely due to the inability to intake food because of oral cavity blistering
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• mice exhibit reduced weight gain during their short lifespan
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• after the initial intake of milk, subsequent intakes were impaired and mice die with empty stomachs
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• as revealed by a dye transfer experiment, skin barrier function is impaired
• mice loss 3.5-fold more water than control Plec1tm4Gwi homozygotes through transepidermal water loss
• following mechanical stress (tape stripping), mice exhibit blistering and dye penetration indicative of induced lesions that were not observed in control Plec1tm4Gwi homozygotes
|
|
• as revealed by a dye transfer experiment, skin barrier function is impaired
• mice loss 3.5-fold more water than control Plec1tm4Gwi homozygotes through transepidermal water loss
• following mechanical stress (tape stripping), mice exhibit blistering and dye penetration indicative of induced lesions that were not observed in control Plec1tm4Gwi homozygotes
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• mice exhibit microblisters and large blisters (up to 1 cm2) on the upper extremities and in the armpits
• blisters form between the dermis and superficial epidermal layers
• blisters form in the oral cavity on the palates and tongue
• however, no blisters are found on the proximal esophagus
• mice subjected to skin mechanical stress form blisters while control Plec1tm4Gwi homozygotes do not
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• mice exhibit microlesions at various stages of wound healing
• following mechanical stress (tape stripping), mice exhibit blistering and dye penetration indicative of induced lesions that were not observed in control Plec1tm4Gwi homozygotes
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• mice exhibit severe skin detachment on the fore- and hindlimb, occasionally around the mouth and nasal cavities, and rarely aplasia cutis (absence of skin)
• mice have fragile skin that is susceptible to mechanical stress injuries such as blisters and microlesions
• however, epidermal stratification and differentiation are normal as is keratinocyte proliferation and survival
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|
|
| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
|
• decreased survival rates starting at 6 months of age
|
| N |
• no abnormal muscle phenotype in early months of life
• extensor digitorum longus is normal at 16 months of age
|
|
• focal disorganization of contractile apparatus
|
|
• mitochondria lose association with Z-discs of muscle fibers
• mitochondria found in focal aggregates in sarcoplasm and subsarcolemmal regions
• numbers of mitochondria in muscle fibers is reduced
|
|
• soleus muscle appears pale at 8 weeks of age and pathological changes are seen at 6 months
|
|
• detached from the contractile apparatus in the soleus and the diaphragm
• 61% of fibers with focal detachments at 8 weeks
|
|
• inner structure of fibers disorganized
• eosinophylic inclusions under the sarcolemma
|
|
• numerous necrotic fibers in the soleus at 8 weeks
|
|
• hypertrophic and split fibers in both the soleus and diaphragm in older mice
|
|
• numerous centrally nucleated fibers in the soleus at 8 weeks
• at 3 months, 35% of fibers in exercised mice are centrally nucleated (45% at 12 months)
|
|
• slight reduction in total number of fibers
|
|
• loss of muscle mass observed in some mice at 16 months of age
|
|
• atrophic fibers in soleus at 1 year
|
| N |
• no heart pathologies at 12 months
• no dilated or hypertrophic cardiomyopathies at 16 months of age
|
|
• increased connective tissue at 16 months of age
|
|
• focal disorganization of contractile apparatus
|
|
• mitochondria lose association with Z-discs of muscle fibers
• mitochondria found in focal aggregates in sarcoplasm and subsarcolemmal regions
• numbers of mitochondria in muscle fibers is reduced
|
|
• numerous necrotic fibers in the soleus at 8 weeks
|
|
• decreased endurance during voluntary wheel running
|
|
• decreased endurance during voluntary wheel running
|
|
• soleus muscle appears pale at 8 weeks of age and pathological changes are seen at 6 months
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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 04/23/2024 MGI 6.23 |
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