Phenotypes Associated with This Genotype

Genotype
MGI:5319194

• Allelic Composition: Tg(Myh6-FKBP1A)4Shou/0
• Genetic Background: involves: C3HeB/FeJ * DBA/2J

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

mortality/aging

lethality, incomplete penetrance ( J:183588 )

• some mice die suddenly, predominantly between 4 - 8 weeks of age

cardiovascular system

increased heart left atrium size ( J:183588 )

• at 6 - 8 weeks of age

increased heart right atrium size ( J:183588 )

• at 6 - 8 weeks of age

decreased heart rate ( J:183588 )

• bradyarrhythmia

irregular heartbeat ( J:183588 )

• bradyarrhythmia

abnormal impulse conducting system conduction ( J:183588 )

• significant prolongations of the atrial-His and His-ventricular intervals in perfused hearts

• slower conduction velocity and marked AP prolongation in the left ventricular epicardium of isolated hearts

atrioventricular block ( J:183588 )

• most (82 of 105) mice display some degree of atrioventricular conduction block

• significant prolongations of the pacing cycle length at which Wenckebach-type AV block occurred and the effective refractory period of the AV node

abnormal heart electrocardiography waveform feature ( J:183588 )

• prolonged PP and PQ intervals

prolonged P wave ( J:183588 )

• prolonged duration

prolonged PQ interval ( J:183588 )

prolonged QRS complex duration ( J:183588 )

abnormal myocardial fiber physiology ( J:183588 )

• mean peak macroscopic voltage-gated Na+ current densities are dramatically reduced and recovery of currents from inactivation is delayed in ventricular cardiomyocytes

• slight increase in the sustained K+ current density

• decrease in peak L-type Ca2+ currents and a proportional reduction in the amplitude of the peak intracellular calcium transient