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Phenotypes Associated with This Genotype
Genotype
MGI:4818886
Allelic
Composition
Tg(Myh6-PRKAG2*T400N)1Feah/0
Genetic
Background
involves: FVB/N
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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 large vacuoles filled with glycogen unlike in wild-type cells
• beginning at 1 week of age
• 4 times wild-type at 4 weeks
• beginning at 4 weeks, mice exhibit increased left ventricular anterior wall thickness (LVAWT) compared with wild-type mice
• LVAWT reaches maximum at 8 weeks and progressively declines until 20 weeks
• beginning at 12 weeks, left ventricular end diastolic diameter is increased compared to in wild-type mice
• beginning at 12 weeks, mice exhibit decreased fractional shortening compared with wild-type mice
• at 20 weeks, fractional shortening is reduced one third compared to in wild-type mice
• mice exhibit ventricular preexcitation indicated by the presence of delta waves, a short PR interval, and a wide QRS compared with wild-type mice
• following ischemia-reperfusion injury, mice exhibit a larger area of necrosis, increased apoptosis, and greater neutrophil infiltration compared with similarly treated wild-type mice
• following ischemia-reperfusion injury

homeostasis/metabolism
• following ischemia-reperfusion injury, mice exhibit a larger area of necrosis, increased apoptosis, and greater neutrophil infiltration compared with similarly treated wild-type mice
• following ischemia-reperfusion injury
• between 1 to 8 weeks, myocytes exhibit increased glycogen levels compared with wild-type cells
• however, glycogen levels revert towards wild-type levels by 20 weeks of age

muscle
• myocytes exhibit large vacuoles filled with glycogen unlike in wild-type cells
• beginning at 12 weeks, mice exhibit decreased fractional shortening compared with wild-type mice
• at 20 weeks, fractional shortening is reduced one third compared to in wild-type mice
• between 1 to 8 weeks, myocytes exhibit increased glycogen levels compared with wild-type cells
• however, glycogen levels revert towards wild-type levels by 20 weeks of age

Mouse Models of Human Disease
OMIM ID Ref(s)
Glycogen Storage Disease of Heart, Lethal Congenital 261740 J:162180


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