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Phenotypes Associated with This Genotype
Genotype
MGI:3841175
Allelic
Composition
Tg(Myh6-PRKAG2*N488I)4623Jse/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
mortality/aging
• in response to stress due to cardiac dysfunction (J:128648)
• in response to stress due to cardiac dysfunction (J:128648)
• at 20 to 40 weeks, mice exhibit spontaneous mortality associated with severe left ventricular dysfunction unlike wild-type mice (J:128648)
• all mice die by day 300 (J:128648)
• at 20 to 40 weeks, mice exhibit spontaneous mortality associated with severe left ventricular dysfunction unlike wild-type mice (J:128648)
• all mice die by day 300 (J:128648)

cardiovascular system
• the annulus fibrosis that insulates the atria from the ventricles is thinned, stretched, and disrupted unlike in wild-type mice (J:128648)
• the annulus fibrosis that insulates the atria from the ventricles is thinned, stretched, and disrupted unlike in wild-type mice (J:128648)
• distended myocytes are filled with glycogen unlike in wild-type mice (J:128648)
• distended myocytes are filled with glycogen unlike in wild-type mice (J:128648)
• at 8 to 10 weeks and 20 weeks (J:128648)
• at 8 to 10 weeks and 20 weeks (J:128648)
• left ventricular wall (J:128648)
• left ventricular wall (J:128648)
• at 8 to 10 weeks and 20 weeks, fractional shortening is decreased and left ventricular end-diastolic diameter is increased compared to in wild-type mice (J:128648)
• at 8 to 10 weeks and 20 weeks, fractional shortening is decreased and left ventricular end-diastolic diameter is increased compared to in wild-type mice (J:128648)
• mice exhibit spontaneous episodes of sinus bradycardia and various escape rhythms, including paroxysmal supraventricular tachycardia and atrial fibrillation unlike in wild-type mice (J:128648)
• mice exhibit spontaneous episodes of sinus bradycardia and various escape rhythms, including paroxysmal supraventricular tachycardia and atrial fibrillation unlike in wild-type mice (J:128648)
• at 8 to 10 weeks, mice exhibit decreased heart rate compared to in wild-type mice (J:128648)
• mice exhibit severe sinus bradycardia before death unlike wild-type mice (J:128648)
• however, heart rate at 20 weeks is normal (J:128648)
• at 8 to 10 weeks, mice exhibit decreased heart rate compared to in wild-type mice (J:128648)
• mice exhibit severe sinus bradycardia before death unlike wild-type mice (J:128648)
• however, heart rate at 20 weeks is normal (J:128648)
• mice exhibit preexcitation unlike in wild-type mice (J:128648)
• mice exhibit preexcitation unlike in wild-type mice (J:128648)
• PR interval is shortened in 50% of mice (J:128648)
• PR interval is shortened in 50% of mice (J:128648)
• in response to stress, mice exhibit syncope that led to death that is associated with severe and persistent sinus brachycardia unlike wild-type mice (J:128648)
• in response to stress, mice exhibit syncope that led to death that is associated with severe and persistent sinus brachycardia unlike wild-type mice (J:128648)

homeostasis/metabolism
• in response to stress, mice exhibit syncope that led to death that is associated with severe and persistent sinus brachycardia unlike wild-type mice (J:128648)
• in response to stress, mice exhibit syncope that led to death that is associated with severe and persistent sinus brachycardia unlike wild-type mice (J:128648)
• cardiac glycogen levels are higher than in wild-type mice (J:128648)
• cardiac glycogen levels are higher than in wild-type mice (J:128648)

muscle
• distended myocytes are filled with glycogen unlike in wild-type mice (J:128648)
• distended myocytes are filled with glycogen unlike in wild-type mice (J:128648)
• at 8 to 10 weeks and 20 weeks, fractional shortening is decreased and left ventricular end-diastolic diameter is increased compared to in wild-type mice (J:128648)
• at 8 to 10 weeks and 20 weeks, fractional shortening is decreased and left ventricular end-diastolic diameter is increased compared to in wild-type mice (J:128648)

Mouse Models of Human Disease
OMIM ID Ref(s)
Cardiomyopathy, Familial Hypertrophic, 6; CMH6 600858 J:128648


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
01/26/2016
MGI 6.02
The Jackson Laboratory