Mouse Genome Informatics
tg
    Tg(Myh6-PRKAG2*T400N)1Feah/0
involves: FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
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

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