Mouse Genome Informatics
ht
    Scn5atm1Pec/Scn5a+
involves: Swiss
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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 IDRef(s)
Long QT Syndrome 3; LQT3 603830 J:71542