Analysis Tools
mortality/aging
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• mice are born alive but exhibit postnatal death around 3rd week of age and are all dead by 9th week
• males die sooner than females, around 6 weeks of age
• mice show signs of distress 1-2 days before death
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growth/size/body
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• at 25 days of age, both males and females show a significant increase in heart weight/body weight ratios relative to controls
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• surviving males show a significant decrease in body weight at 4 and 5 weeks of age relative to controls
• in contrast, females show normal body weight up to 6 weeks of age
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cardiovascular system
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• at 25 days of age, the area and perimeter of the area between cardiac muscle fibers are increased relative to controls
• cardiac fibers show altered sarcomeres with irregular Z-discs and unidentifiable M-lines, disarrayed thin filaments, and distorted intercalated discs
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• at 25 days of age, cardiac fibers display less convoluted intercalated discs (ICDs) with reduced electron-dense staining, indicating less ICD component proteins
• expression of two key ICD genes, beta-catenin and Connexin43 (components of fascia adherens and gap junctions, respectively) is reduced
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• at 25 days of age, both males and females show a significant increase in heart weight/body weight ratios relative to controls
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• at 25 days of age, all mice exhibit thin ventricular walls during both systolic and diastolic periods
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• at 25 days of age, all mice exhibit dilated heart ventricular lumens
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• at 25 days of age, mutant hearts display dilated cardiomyopathy defects with a significant increase in expression of the heart hypertrophy and heart failure biomarkers, atrial natriuretic factor (ANF) and
brain natriuretic peptide (BNP), before detection of heart failure
• however, no increase in cardiac fibrosis is observed
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• at 25 days of age, mice display markedly reduced ejection fraction and fractional shortening values relative to controls
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• at 25 days of age, mice display delayed electrical repolarization of the ventricles (as shown by lengthened QTc value), indicating ventricular arrhythmia
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• at 25 days of age, mice display a lengthened corrected QT interval (QTc) value relative to controls
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muscle
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• at 25 days of age, the area and perimeter of the area between cardiac muscle fibers are increased relative to controls
• cardiac fibers show altered sarcomeres with irregular Z-discs and unidentifiable M-lines, disarrayed thin filaments, and distorted intercalated discs
|
|
• at 25 days of age, cardiac fibers display less convoluted intercalated discs (ICDs) with reduced electron-dense staining, indicating less ICD component proteins
• expression of two key ICD genes, beta-catenin and Connexin43 (components of fascia adherens and gap junctions, respectively) is reduced
|
|
• at 25 days of age, mutant hearts display dilated cardiomyopathy defects with a significant increase in expression of the heart hypertrophy and heart failure biomarkers, atrial natriuretic factor (ANF) and
brain natriuretic peptide (BNP), before detection of heart failure
• however, no increase in cardiac fibrosis is observed
|
|
• at 25 days of age, mice display markedly reduced ejection fraction and fractional shortening values relative to controls
|
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• at 25 days of age, sarcomeres are shortened and disorganized in the myocardium
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• at 25 days of age, M-lines are unrecognizable in the myocardium
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• at 25 days of age, Z-discs are disorganized in the myocardium
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behavior/neurological
trunk curl
(
J:233669
)
|
• mice exhibit curling up 1-2 days prior to death
|
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• mice exhibit less movement 1-2 days prior to death
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respiratory system
|
• mice exhibit dyspnea 1-2 days prior to death
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| dilated cardiomyopathy | DOID:12930 |
OMIM:PS115200 |
J:233669 | |
