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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Juptm1.1Shou
targeted mutation 1.1, Weinian Shou
MGI:5296511
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
cn1
Juptm1.1Shou/Juptm1.1Shou
Tg(Myh6-cre)2182Mds/0
involves: 129 * C57BL/6J MGI:5296512


Genotype
MGI:5296512
cn1
Allelic
Composition
Juptm1.1Shou/Juptm1.1Shou
Tg(Myh6-cre)2182Mds/0
Genetic
Background
involves: 129 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Juptm1.1Shou mutation (0 available); any Jup mutation (139 available)
Tg(Myh6-cre)2182Mds mutation (3 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutants suddenly die starting at 1 month of age, with an average life-span of 4.6 months

cardiovascular system
• mutants exhibit severe liver congestion
• cardiomyocytes are hypertrophic, showing an increase in cross-sectional area
• hearts show loss of desmosomes, however adherens junctions are normal
• myocardial calcification is seen in 2-month old ventricles
• heart is enlarged at 2 months of age
• hearts exhibit regional dysplasia of ventricular walls and ventricular aneurysms
• cardiac fibrosis is seen in atria and ventricles; fibrosis ranges from moderate to severe
• ECG shows compromised systolic function of the left ventricle
• fragmented diastolic potentials are seen in right ventricle
• hearts show reduced fractional shortening and ejection fraction
• at 1 and 2 months of age, mutants exhibit spontaneous non-sustained ventricular tachycardia
• sustained monomorphic ventricular tachycardias are induced in mutants by bust or programmed ventricular stimulation in 6 of 7 mutants but not in controls
• mutants exhibit intracardiac bipolar electrograms during ventricular tachycardia
• in Langendorff-perfused hearts, conduction velocity max and min are reduced
• at 1 month of age, mutants exhibit complex electrophysiological abnormalities, with low amplitude of the QRS complex, prolonged PR interval and spontaneous non-sustained ventricular tachycardia
• by 2 months of age, the amplitude of the P-wave is higher, amplitude of QRS complex is lower, the PR interval is prolonged, and frequent spontaneous ventricular ectopic beats are seen
• at 1 and 2 months of age, mutants exhibit prolonged PR interval
• by 2 months of age, the amplitude of the P-wave is higher
• ECG shows decreased QRS complex amplitude at P18, 1 month and 2 months of age
• 18 day old mutants exhibit extensive cardiomyocyte death, including apoptisis and necrosis
• mutants show rapid and progressive development of cardiomyopathy; hearts appear normal at P12 but by P18, show signs of fibrosis and heart enlargement by 1 month

liver/biliary system
• mutants exhibit severe liver congestion

muscle
• cardiomyocytes are hypertrophic, showing an increase in cross-sectional area
• hearts show loss of desmosomes, however adherens junctions are normal
• hearts show reduced fractional shortening and ejection fraction
• 18 day old mutants exhibit extensive cardiomyocyte death, including apoptisis and necrosis
• mutants show rapid and progressive development of cardiomyopathy; hearts appear normal at P12 but by P18, show signs of fibrosis and heart enlargement by 1 month
• 18 day old mutants exhibit extensive cardiomyocyte death, including apoptisis and necrosis

cellular
• 18 day old mutants exhibit extensive cardiomyocyte death, including apoptisis and necrosis





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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory