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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Scn5atm1Pec
targeted mutation 1, Peter Carmeliet
MGI:2388158
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Scn5atm1Pec/Scn5atm1Pec involves: Swiss MGI:3621907
ht2
Scn5atm1Pec/Scn5a+ involves: Swiss MGI:3622652


Genotype
MGI:3621907
hm1
Allelic
Composition
Scn5atm1Pec/Scn5atm1Pec
Genetic
Background
involves: Swiss
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Scn5atm1Pec mutation (0 available); any Scn5a mutation (41 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging

cardiovascular system




Genotype
MGI:3622652
ht2
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 (41 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





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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
07/19/2016
MGI 6.04
The Jackson Laboratory