Phenotypes Associated with This Genotype

Genotype
MGI:5477914

• Allelic Composition: Gja1^tm10Kwi/Gja1^tm10Kwi; Tg(Myh6-icre/ERT2)#Wet/0
• Genetic Background: involves: 129P2/OlaHsd * C57BL/6 * FVB/N

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

mortality/aging

premature death ( J:194957 )

• all mice die 16 to 21 days after tamoxifen-treatment

cardiovascular system

cardiovascular system phenotype ( J:194957 )

N • mice exhibit normal heart beating frequency and PQ duration

ventricular tachycardia ( J:194957 )

• sustained polymorphic ventricular tachycardia degenerating into ventricular fibrillation in tamoxifen-treated mice

• tamoxifen-treated mice exhibit sustained ventricular tachycardia or ventricular fibrillation induced by fixed-rate epicardial stimulation of the ventricle unlike control mice

irregular heartbeat ( J:194957 )

• non-sustained episodes of monomorphic and polymorphic ventricular arrhythmias 15 to 17 days after tamoxifen-treatment

ventricular fibrillation ( J:194957 )

• in tamoxifen-treated mice

atrioventricular block ( J:194957 )

• in tamoxifen-treated mice

prolonged QRS complex duration ( J:194957 )

• 2.1-fold in tamoxifen-treated mice

abnormal myocardial fiber physiology ( J:194957 )

• cardiomyocytes form tamoxifen-treated mice exhibit reduced average peak inward sodium current density, prolonged time course of inward sodium channel inactivation, reduced average peak outward current density and increased action potential duration at 50 and 70% depolarization compared with control cells

• however, cardiomyocytes exhibit normal voltage-dependent inactivation, time course of recovery from inactivation, density of inward rectifier current, resting potential, action potential amplitude and action potential duration at 90% repolarization