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Phenotypes Associated with This Genotype
Genotype
MGI:5575893
Allelic
Composition
Tg(Myh6-KCNQ1_i2)H02Desc/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
• mice show ECG anomalies indicative of decreased heart rate
• 23% of 4-6 week old mice exhibit a Wenckebach phenomenon, suggesting an intranodal conduction defect
• prolonged AH interval, with a normal HV interval, leading to a non conducted A wave, indicating atrioventricular block originating from an intranodal conduction defect and not a His-bundle conduction defect
• prolonged AH interval, with a normal HV interval, leading to a non conducted A wave, indicating AV block originating from an intranodal conduction defect
• ECG shows prolonged action potential duration and reduced outward K+ current
• QT interval is longer than in wild-type mice or in transgenic lines H08 or H05
• however, QRS duration is normal
• the rapid component of the T wave is drastically 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
• the P wave often superposes on the terminal phase of the previous T wave due to QT and PR lengthening
• longer P wave duration than wild-type mice or transgenic lines H08 and H05

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
long QT syndrome 1 DOID:0110644 OMIM:192500
J:132483


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
06/12/2024
MGI 6.13
The Jackson Laboratory