mortality/aging
• mice die within 4 weeks
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• mice die within 4 weeks
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cardiovascular system
• mice show abnormalities in pectinate muscles in atrial appendages
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• cultured attached cardiomyocytes are smaller than similarly treated wild-type cells
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• significantly thinner compact myocardium at P1
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• at P1, myocardium exhibit widened gaps of intercalated disks with disarrayed sarcomeres and distorted Z lines compared to in wild-type mice
• at P10, membranes of the intercalated disk are highly convoluted and gaps are widened unlike in wild-type mice
• the myocardium exhibits disruption of cell-cell adhesion compared to in wild-type mice
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• at P20
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• hearts are abnormally shaped with a bulge at the base of the right ventricle at P20
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• hearts are rounder at P1
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• mice exhibit disorganized trabeculae that fail to fuse and incorporate into compact myocardium unlike in wild-type mice
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• at P1 and P20
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• at P20, the ventricular wall exhibits irregular thickness with a thickened right ventricular free wall and thin left ventricular free wall and septum compared to in wild-type mice
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• at P20, hearts show fibrosis, esp. in the ventricular septum and within thinned regions of ventricular myocardium
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• few cardiomyocytes attack to a plate coated in fibronectin, laminin, and collagen compared with wild-type cells
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• progressive, resulting in death
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homeostasis/metabolism
• at P20, a large intracardiac thrombus is frequently detected in the left atria
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liver/biliary system
respiratory system
growth/size/body
• gradual
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muscle
• mice show abnormalities in pectinate muscles in atrial appendages
|
• cultured attached cardiomyocytes are smaller than similarly treated wild-type cells
|
• significantly thinner compact myocardium at P1
|
• at P1, myocardium exhibit widened gaps of intercalated disks with disarrayed sarcomeres and distorted Z lines compared to in wild-type mice
• at P10, membranes of the intercalated disk are highly convoluted and gaps are widened unlike in wild-type mice
• the myocardium exhibits disruption of cell-cell adhesion compared to in wild-type mice
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