Phenotypes Associated with This Genotype

Genotype
MGI:5474053

• Allelic Composition: Tbx5^tm1.2Jse/Tbx5^tm1.2Jse; Tg(Kcne1-cre/ERT2)1Imos/0
• Genetic Background: involves: CD-1

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

abnormal induced morbidity/mortality ( J:190765 )

• sudden death occurring within 5 weeks of tamoxifen administration (at 6 weeks) is observed

• one mouse that developed spontaneous ventricular tachycardia died suddenly prior to any electrophysiological testing

premature death ( J:190765 )

• following tamoxifen treatment starting at 6 weeks, animals demonstrate sudden death as soon as 5 weeks post administration

cardiovascular system

cardiovascular system phenotype ( J:190765 )

N • mutants have similar contractile function to controls; mice display immediate recovery of normal cardiac function after episodes of spontaneous ventricular tachycardia

ventricular tachycardia ( J:190765 )

• displayed by some tamoxifen-treated mutants; in ambulatory studies and electrophysiologic (EP) studies, spontaneous monomorphic ventricular tachycardia episodes are observed in some mice

• mice show significantly increased susceptibility to ventricular tachycardia after burst stimulation in EP studies

irregular heartbeat ( J:190765 )

• significant arrhythmias are observed in tamoxifen-treated mutants, including Mobitz type II second-degree AV block (indicating infranodal conduction system disease)

ventricular premature beat ( J:190765 )

• premature ventricular contractions (PVCs) numbering more than 100 per 24 hour period are detected in more than half of mutant mice

abnormal impulse conducting system conduction ( J:190765 )

• severe conduction slowing is observed in tamoxifen-treated animals 4-5 weeks after tamoxifen administration; the PR interval and QRS duration are significantly increased compared to controls

abnormal atrioventricular bundle conduction ( J:190765 )

• slowed conductance through the His bundle is detected; non-ventricular conduction system (VCS) function is not altered

atrioventricular block ( J:190765 )