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Phenotypes Associated with This Genotype
Genotype
MGI:3841180
Allelic
Composition
Tg(Myh6-tTA)55Rbns/0
Tg(Myh6/tetO-PRKAG2*N488I)1Chib/0
Genetic
Background
Not Specified
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
• the anulus fibrosus that separates the atria from ventricles is discontinuous compared to in wild-type mice
• the anulus fibrosus in mice treated with doxycycline between 4 and 16 weeks is disrupted and disorganized without vacuolization of the septal and left ventricular wall myocytes
• however, the anulus fibrosus is intact in mice treated early with doxycycline (prenatal to 8 weeks)
• unlike in wild-type mice, vacuoles are present in the myocytes of the right ventricle in mice treated with doxycycline between 4 and 16 weeks
• hypertrophic at 8 weeks of age
• heart weight is increased compared to in wild-type mice
• however, doxycycline treatment reduces heart weight 30% compared to in untreated mice
• unlike in wild-type mice, vacuoles are present in the myocytes of the right ventricle in mice treated with doxycycline between 4 and 16 weeks
• mice exhibit increased ventricular wall thickness compared to in wild-type mice
• early doxycycline treatment (prenatally to 8 weeks of age) delays myocardial wall thickening
• however, doxycycline treatment reduces ventricular wall thickness irregardless of treatment time
• at 16 weeks, mice exhibit progressive systolic dysfunction indicated by increased left ventricle end diastolic diameter and decreased fractional shortening unlike wild-type mice
• however, doxycycline treatment ameliorates myocardial dysfunction
• mice exhibit decreased heart rates compared to in wild-type mice regardless of doxycycline treatment
• 50% of mice frequently exhibit supraventricular tachycardia
• however, mice treated early (prenatal to 8 weeks) with doxycycline do not develop supraventricular tachycardia
• mice exhibit preexcitation unlike in wild-type mice
• early doxycycline treatment (prenatally to 8 weeks of age) delays myocardial dysfunction
• doxycycline-treatment between 4 and 16 weeks results in a decline of preexcitation from 50% to 11% and is accompanied by atrial pacing
• doxycycline treatment after 8 weeks does not cause loss of preexcitation or alter the electrical properties of accessory pathways
• however, prenatal or early treatment with doxyxycline through 8 weeks of age prevents preexcitation while late treatment (after 20 weeks) improves cardiac conduction system function
• however, mice exhibit normal cardiac conduction velocity regardless of doxycycline treatment
• at 4 weeks, the PR interval is shortened in 50% to 60% of mice compared to in wild-type mice
• AV conduction properties are 50% prolonged compared to in wild-type mice
• 1 of 8 mice exhibited intermittent complete AV block
• early doxycycline suppression (prenatally to 8 weeks of age) does not improve sinus and atrioventriular node abnormalities
• at 4 weeks, 50% to 60% of mice exhibit prolonged, slurred-upstroke QRS complexes unlike in wild-type mice
• 30% of mice exhibit sinoatrial exit block unlike in wild-type mice
• early doxycycline suppression (prenatally to 8 weeks of age) does not improve sinus and atrioventriular node abnormalities

homeostasis/metabolism
• myocardial glycogen content is 30-fold higher than in wild-type mice
• however, doxycycline treatment reduces myocardial glycogen content

muscle
• unlike in wild-type mice, vacuoles are present in the myocytes of the right ventricle in mice treated with doxycycline between 4 and 16 weeks
• at 16 weeks, mice exhibit progressive systolic dysfunction indicated by increased left ventricle end diastolic diameter and decreased fractional shortening unlike wild-type mice
• however, doxycycline treatment ameliorates myocardial dysfunction
• myocardial glycogen content is 30-fold higher than in wild-type mice
• however, doxycycline treatment reduces myocardial glycogen content

Mouse Models of Human Disease
OMIM ID Ref(s)
Glycogen Storage Disease of Heart, Lethal Congenital 261740 J:145090


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last database update
05/17/2016
MGI 6.03
The Jackson Laboratory