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Phenotypes Associated with This Genotype
Genotype
MGI:3589459
Allelic
Composition
Lmnatm3Stw/Lmnatm3Stw
Genetic
Background
involves: 129S1/Sv * 129S4/SvJaeSor * C57BL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmnatm3Stw mutation (0 available); any Lmna mutation (61 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• homozygotes generally die between 12 - 14 weeks of age with none surviving past 16 weeks of age (J:100393)
• in at least 3 homozygotes death resulted from bradyarrhythmia (J:100393)
• homozygotes generally die between 12 - 14 weeks of age with none surviving past 16 weeks of age (J:100393)
• in at least 3 homozygotes death resulted from bradyarrhythmia (J:100393)

growth/size/body
• homozygotes display decreased weight gain starting around 4 weeks of age (J:100393)
• homozygotes display decreased weight gain starting around 4 weeks of age (J:100393)

cardiovascular system
• loss of sarcomere organization is seen in cardiomyocytes and nuclear defects are seen (J:100393)
• loss of sarcomere organization is seen in cardiomyocytes and nuclear defects are seen (J:100393)
• degeneration is associated with decreased fiber size, fragmentation, increased granular cytoplasm with loss of striation, and occasional vacuolation (J:100393)
• degeneration is associated with decreased fiber size, fragmentation, increased granular cytoplasm with loss of striation, and occasional vacuolation (J:100393)
• dilation of the chambers is accompanied by thinning of the chamber walls (J:100393)
• dilation of the chambers is accompanied by thinning of the chamber walls (J:100393)
• increased cardiac interstitial fibrosis is seen at 9 weeks of age; however, no increase in apoptosis is detected (J:100393)
• increased cardiac interstitial fibrosis is seen at 9 weeks of age; however, no increase in apoptosis is detected (J:100393)
• increased levels of atrial natriuretic peptide and B-type natriuretic peptide are seen (J:100393)
• increased levels of atrial natriuretic peptide and B-type natriuretic peptide are seen (J:100393)
• progressive decrease in ventricular fractional shortening is seen starting at 8 weeks of age and left ventricular end-diastolic dimension is significantly larger compared to controls when normalized to body weight (J:100393)
• progressive decrease in ventricular fractional shortening is seen starting at 8 weeks of age and left ventricular end-diastolic dimension is significantly larger compared to controls when normalized to body weight (J:100393)
• continuous electrocardiographic monitoring revealed periods of extreme bradycardia and excessive diurnal variation in heart rate in 8 week old mutants (J:100393)
• continuous electrocardiographic monitoring revealed periods of extreme bradycardia and excessive diurnal variation in heart rate in 8 week old mutants (J:100393)
• continuous electrocardiographic monitoring revealed a variety of conduction abnormalities including 2 mice with progressive prolongations of the PR interval (J:100393)
• continuous electrocardiographic monitoring revealed a variety of conduction abnormalities including 2 mice with progressive prolongations of the PR interval (J:100393)

behavior/neurological
• just prior to death homozygotes display reduced activity (J:100393)
• just prior to death homozygotes display reduced activity (J:100393)

muscle
• loss of sarcomere organization is seen in cardiomyocytes and nuclear defects are seen (J:100393)
• loss of sarcomere organization is seen in cardiomyocytes and nuclear defects are seen (J:100393)
• degeneration is associated with decreased fiber size, fragmentation, increased granular cytoplasm with loss of striation, and occasional vacuolation (J:100393)
• degeneration is associated with decreased fiber size, fragmentation, increased granular cytoplasm with loss of striation, and occasional vacuolation (J:100393)
• increased levels of atrial natriuretic peptide and B-type natriuretic peptide are seen (J:100393)
• increased levels of atrial natriuretic peptide and B-type natriuretic peptide are seen (J:100393)
• progressive decrease in ventricular fractional shortening is seen starting at 8 weeks of age and left ventricular end-diastolic dimension is significantly larger compared to controls when normalized to body weight (J:100393)
• progressive decrease in ventricular fractional shortening is seen starting at 8 weeks of age and left ventricular end-diastolic dimension is significantly larger compared to controls when normalized to body weight (J:100393)
• moderate degeneration of cardiac muscle but only very mild degeneration of skeletal muscle is seen (J:100393)
• moderate degeneration of cardiac muscle but only very mild degeneration of skeletal muscle is seen (J:100393)

integument
• just prior to death homozygotes appear slightly disheveled (J:100393)
• just prior to death homozygotes appear slightly disheveled (J:100393)

Mouse Models of Human Disease
OMIM ID Ref(s)
Cardiomyopathy, Dilated, 1A; CMD1A 115200 J:100393


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory