Mouse Genome Informatics
tg1
    Tg(Myh6-PRKAG2*R302Q)#Rro/0
involves: FVB
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
cardiovascular system
• myocytes exhibit multiple nuclei with great variation in size, indicating hypertrophy
• large vacuoles are abundant throughout the myocardium of the right and left ventricles
• exhibit muscle bundles that extend from the atrium to the ventricle in the region of the fibrous body
• heart weight is nearly 3x that of nontransgenic controls
• hypertrophic cardiomyoctyes
• posterior ventricular wall thickness at 2 months of age is increased and exhibit an increase in end-diastolic diameter and end-systolic diameter with decreased systolic function
• atrial electrical stimulation induced supraventricular tachycardia in 6 of 12 mutants, consistent with orthodromic atrioventricular (AV) reentrant tachycardia characterized by antegrade conduction via the AV node and retrograde conduction over the accessory pathway
• atrial premature beats and/or tachycardia are observed spontaneously or after atrial electrical stimulation in 58% of mutants, however ventricular electrical stimulation does not induce ventricular arrhythmias
• 21 of 134 show ventricular preexcitation as evidenced by a short PR interval and a wide QRS, and a significant prolongation of the sinus cycle length with normal sinus node recovery times
• exhibit the presence of a functioning accessory pathway bypassing the atrioventricular (AV) node
• baseline ECG recordings of 21 of 134 mutants show a short PR interval
• baseline ECG recordings of 21 of 134 mutants show increased QRS duration with slurred upstroke

homeostasis/metabolism
• exhibit myocytes accumulating increased glycogen in the myocardium of the right and left ventricles

muscle
• myocytes exhibit multiple nuclei with great variation in size, indicating hypertrophy
• large vacuoles are abundant throughout the myocardium of the right and left ventricles
• exhibit muscle bundles that extend from the atrium to the ventricle in the region of the fibrous body
• posterior ventricular wall thickness at 2 months of age is increased and exhibit an increase in end-diastolic diameter and end-systolic diameter with decreased systolic function

Mouse Models of Human Disease
OMIM IDRef(s)
Wolff-Parkinson-White Syndrome 194200 J:108102