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Phenotypes Associated with This Genotype
Genotype
MGI:3622652
Allelic
Composition
Scn5atm1Pec/Scn5a+
Genetic
Background
involves: Swiss
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Scn5atm1Pec mutation (0 available); any Scn5a mutation (43 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 6 of 275 suddenly died around 3-6 months of age, presumably because of fatal arrhythmias

cardiovascular system
• resting heart rate is lower
• some spontaneously develop polymorphous ventricular arrhythmias
• sudden accelerations in heart rate or premature beats cause lengthening of the action potential with early afterdepolarization and trigger arrhythmias
• increase in peak and late sodium current in cardiomyoctes
• ECG recordings show abnormal and variable myocardial propagation of the electrical pulse
• QT interval is prolonged by 55%
• T-wave is more prominent than in wild-type

Mouse Models of Human Disease
OMIM ID Ref(s)
Long QT Syndrome 3; LQT3 603830 J:71542


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
11/29/2016
MGI 6.06
The Jackson Laboratory