Analysis Tools
normal phenotype
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• mice are phenotypically indistinguishable from wild-type controls
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Allele Symbol Allele Name Allele ID |
Hnrnputm1.1Tman targeted mutation 1.1, Tom Maniatis MGI:5648110 |
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| Summary |
4 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• mice are phenotypically indistinguishable from wild-type controls
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
Aberrant cardiomyocyte oranization and contractility in Hnrnputm1.1Tman/Hnrnputm1.1Tman Tg(Ckmm-cre)5Khn/0 hearts
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• although born at normal Mendelian ratios, all mice die abruptly from heart failure around 14 days after birth
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• cardiomyocytes appear disorganized, slightly hypertrophic, and are surrounded by more white space upon H&E staining
• trichrome staining revealed an increased blue signal between cardiomyocytes, suggesting increased expression of the extracellular matrix
• cardiomyocytes show abnormal actin dynamics and shortened sarcomeres
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• overall density of cardiomyocytes is reduced, likely due to heart remodeling
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• cardiomyocytes appear slightly hypertrophic
• however, no major cardiac hypertrophy is observed at P12
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• thinning of the ventricle septum is noted at P14
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• thinning of the ventricle wall is noted at P14
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• mice show progressive dilation of the heart ventricle chambers
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• dilation of the left ventricle chamber is observed as early as P7
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• mice develop severe, lethal dilated cardiomyopathy
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• fractional shortening (FS) is significantly reduced (below 20%) relative to controls (above 60%)
• reduced FS is detected as early as P6, and this decrease progresses rapidly to the time of death
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• ability of sarcomeres to fully relax is impaired in cardiomyocytes
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• at P11, B- and M-mode images of echocardiography show a dramatic increase of left ventricular anterior-to-posterior wall diameter during systole and diastole
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• cardiomyocytes show abnormal actin dynamics and contractility defects
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• at P13, hearts exhibit a slightly higher level of apoptosis than control hearts, as determined by cleaved caspase 3 staining
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• cardiac malfunction starts early after birth and progresses rapidly to cardiac failure
• however, no differences in heart rate are observed from P3 to P11
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• cardiomyocytes appear disorganized, slightly hypertrophic, and are surrounded by more white space upon H&E staining
• trichrome staining revealed an increased blue signal between cardiomyocytes, suggesting increased expression of the extracellular matrix
• cardiomyocytes show abnormal actin dynamics and shortened sarcomeres
|
|
• overall density of cardiomyocytes is reduced, likely due to heart remodeling
|
|
• cardiomyocytes appear slightly hypertrophic
• however, no major cardiac hypertrophy is observed at P12
|
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• mice develop severe, lethal dilated cardiomyopathy
|
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• fractional shortening (FS) is significantly reduced (below 20%) relative to controls (above 60%)
• reduced FS is detected as early as P6, and this decrease progresses rapidly to the time of death
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• ability of sarcomeres to fully relax is impaired in cardiomyocytes
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• at P13, hearts exhibit a slightly higher level of apoptosis than control hearts, as determined by cleaved caspase 3 staining
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• under conditions of muscle relaxation, the lengths of sarcomeres and I bands are markedly reduced in cardiomyocytes
• at P11, the average length of sarcomeres (Z line to Z line) is only 1.68 um versus ~2.24 um in controls
• some regions of sarcomeres lack mitochondria coverage
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• the lengths of I bands are markedly reduced in cardiomyocytes
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• at P13, hearts exhibit a slightly higher level of apoptosis than control hearts, as determined by cleaved caspase 3 staining
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• some regions of sarcomeres lack mitochondria coverage
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• amplitude of heart contraction is significantly reduced relative to that in control hearts
• analysis of calcium cycling and heart contraction revealed impaired calcium handling
• hearts reach the maximal calcium level slightly slower than control hearts
• rate of post-contraction calcium decrease is significantly slower than that in controls hearts
• calcium decays almost linearly from the peak signal, in contrast to a two-phase (a rapid drop within the first 20 ms followed by a slower decrease for about 200 ms) reduction in control hearts
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• amplitude of heart contraction is significantly reduced relative to that in control hearts
• analysis of calcium cycling and heart contraction revealed impaired calcium handling
• hearts reach the maximal calcium level slightly slower than control hearts
• rate of post-contraction calcium decrease is significantly slower than that in controls hearts
• calcium decays almost linearly from the peak signal, in contrast to a two-phase (a rapid drop within the first 20 ms followed by a slower decrease for about 200 ms) reduction in control hearts
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• although born at normal Mendelian ratios, all mice die abruptly from heart failure at exactly 10 days after birth
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• mice develop severe, lethal dilated cardiomyopathy
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• mice develop severe, lethal dilated cardiomyopathy
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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 04/16/2024 MGI 6.23 |
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