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Phenotypes Associated with This Genotype
Genotype
MGI:5485272
Allelic
Composition
Tg(Myh6-tTA)6Smbf/0
Tg(tetO-Spp1)5Gad/0
Genetic
Background
involves: C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-tTA)6Smbf mutation (4 available)
Tg(tetO-Spp1)5Gad mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mutants show a large increase in mortality occurring between the 8th and 15th week after birth, with a half-life of 12 weeks

cardiovascular system
• cellular organization in hearts is altered after 11 weeks of age, with evidence of myocyte loss and interstitial cell infiltratio
• the number of apoptotic cells in the hear is increased at 11 weeks of age
• at 11 weeks of age, but not 6 weeks, heart left ventricle is severely dilated
• at 11 weeks of age, the left ventricle free wall is thinner during systole than in controls, suggesting the development of a hypokinetic dilated cardiomyopathy
• mutants exhibit systolic dysfunction at 11 weeks of age, indicated by reduced left ventricle fractional shortening
• ECG intervals during the last ECG recording before death show differences in PQ, QRS, QT, and QTc but not of RR
• mice that survive exhibit ECG parameters with normal values, with a moderate prolongation of PQ and QRS intervals
• mice that die during the 15th week, show complete atrioventricular block, with dissociation between the sinus and idioventricular escape rhythms
• at 11 weeks of age
• at 11 weeks of age, mutants show a moderately prolonged QRS interval
• at 11 weeks of age, ECGs show an abnormal repolarization with disappearance of the early positive phase of the T wave commonly seen in controls
• at 11 weeks of age, the T-wave duration is prolonged
• however, the QT interval is not prolonged
• hearts are infiltrated by a large number of interstitial cells; about 50% of infiltrating cells express panleukocyte CD45 marker

immune system
• hearts are infiltrated by a large number of interstitial cells; about 50% of infiltrating cells express panleukocyte CD45 marker

muscle
• at 11 weeks of age, the left ventricle free wall is thinner during systole than in controls, suggesting the development of a hypokinetic dilated cardiomyopathy
• mutants exhibit systolic dysfunction at 11 weeks of age, indicated by reduced left ventricle fractional shortening

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
dilated cardiomyopathy DOID:12930 OMIM:PS115200
J:194810


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
11/12/2024
MGI 6.24
The Jackson Laboratory