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Phenotypes Associated with This Genotype
Genotype
MGI:5575895
Allelic
Composition
Tg(Myh6-KCNQ1_i2)H05Desc/0
Genetic
Background
involves: FVB
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• cardiac sodium channel remodeling/upregulation that occurs before the onset of cardiac hypertrophy
• cardiac hypertrophy, including increases in myocardial cross-section, with no differences in cell proliferation is seen at E18.5, but not at E14.5
• sodium current recordings show that peak current densities are 30-40% higher in ventricular myocytes, a 30-40% higher conductance, and slower recovery time from inactivation
• while wild-type ventricles have a predominately left-right activation pattern (94%) at E18.5, mutant hearts maintain a left ventricular activation pattern (84%), indicating abnormal ventricular activation pattern during development
• under spontaneous rhythm, mice show an increase in total left ventricular activation time at E18.5 and in adults, but not at E14.5, indicating a delay in the transmission of the electrical impulse
• however, under stimulated contraction, no differences in left ventricular activation time at any age are seen, indicating that electrical impulse transmission through the working myocardium is not altered
• sodium current recordings from ventricular myocytes show a 30-40% higher conductance
• QT interval prolongation, although to a lesser extent than in transgenic lines H08 or H02 (J:132483)
• the rapid component of the T wave is slightly prolonged
• sinus node dysfunction as indicated by a longer intrinsic sinus period under autonomic nervous blockade and an increase in minimum sinus period under isoproterenol infusion, although to a lesser extent than in transgenic lines H08 or H02
• longer P wave duration than in wild-type mice, although to a lesser extent than in transgenic lines H08 or H02
• however, PR interval duration is similar to wild-type mice

mortality/aging
• lower survival rate at 1 year of age
• lower survival rate at 1 year of age

growth/size/body
• cardiac hypertrophy, including increases in myocardial cross-section, with no differences in cell proliferation is seen at E18.5, but not at E14.5

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
long QT syndrome 1 DOID:0110644 OMIM:192500
J:211437


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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
06/12/2024
MGI 6.13
The Jackson Laboratory