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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Lmod2Tn(pb-Act-RFP)1.1Zhu
transposon insertion 1.1, Yuan Zhuang
MGI:5789380
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Lmod2Tn(pb-Act-RFP)1.1Zhu/Lmod2Tn(pb-Act-RFP)1.1Zhu involves: FVB/N MGI:5796293


Genotype
MGI:5796293
hm1
Allelic
Composition
Lmod2Tn(pb-Act-RFP)1.1Zhu/Lmod2Tn(pb-Act-RFP)1.1Zhu
Genetic
Background
involves: FVB/N
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Lmod2Tn(pb-Act-RFP)1.1Zhu mutation (0 available); any Lmod2 mutation (27 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 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

growth/size/body
• at 25 days of age, both males and females show a significant increase in heart weight/body weight ratios relative to controls
• 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

cardiovascular system
• 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, both males and females show a significant increase in heart weight/body weight ratios relative to controls
• at 25 days of age, all mice exhibit thin ventricular walls during both systolic and diastolic periods
• at 25 days of age, all mice exhibit dilated heart ventricular lumens
• 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
• at 25 days of age, mice display delayed electrical repolarization of the ventricles (as shown by lengthened QTc value), indicating ventricular arrhythmia
• at 25 days of age, mice display a lengthened corrected QT interval (QTc) value relative to controls

muscle
• 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
• at 25 days of age, sarcomeres are shortened and disorganized in the myocardium
• at 25 days of age, M-lines are unrecognizable in the myocardium
• at 25 days of age, Z-discs are disorganized in the myocardium

behavior/neurological
• mice exhibit curling up 1-2 days prior to death
• mice exhibit less movement 1-2 days prior to death

respiratory system
• mice exhibit dyspnea 1-2 days prior to death

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
dilated cardiomyopathy DOID:12930 OMIM:PS115200
J:233669





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last database update
10/29/2024
MGI 6.24
The Jackson Laboratory