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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(Myh6-PRKAG2*R302Q)#Rro
transgene insertion #, Robert Roberts
MGI:3688892
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(Myh6-PRKAG2*R302Q)#Rro/0 involves: FVB MGI:3689004


Genotype
MGI:3689004
tg1
Allelic
Composition
Tg(Myh6-PRKAG2*R302Q)#Rro/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
• 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
• exhibit myocytes accumulating increased glycogen in the myocardium of the right and left ventricles

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





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last database update
11/29/2016
MGI 6.06
The Jackson Laboratory