Phenotypes Associated with This Genotype

Genotype
MGI:6501732

• Allelic Composition: Tg(Myh6-JUP*)1Dpju/0
• Genetic Background: FVB/N-Tg(Myh6-JUP*)1Dpju

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:235770 )

• mice die at approximately 1 year of age

cardiovascular system

cardiac fibrosis ( J:235770 )

• mice exhibit focal areas of fibrosis

• mice treated daily with SB216763 at 6 months of age do not show cardiac fibrosis

ventricular tachycardia ( J:235770 )

• mice exhibit frequent short runs of ventricular tachycardia

ventricular premature beat ( J:235770 )

• mice exhibit frequent ventricular ectopy

• SB216763 treatment reduces ventricular ectopy

heart inflammation ( J:235770 )

• hearts exhibit focal areas of inflammation

• mice treated daily with the GSK3B inhibitor SB216763 at 6 months of age do not show cardiac inflammation

immune system

heart inflammation ( J:235770 )

• hearts exhibit focal areas of inflammation

• mice treated daily with the GSK3B inhibitor SB216763 at 6 months of age do not show cardiac inflammation

muscle

increased cardiomyocyte apoptosis ( J:235770 )

• hearts exhibit increased apoptotic cardiomyocytes

• mice treated daily with SB216763 at 6 months of age show elimination of cardiomyocyte apoptosis

cellular

increased cardiomyocyte apoptosis ( J:235770 )

• hearts exhibit increased apoptotic cardiomyocytes

• mice treated daily with SB216763 at 6 months of age show elimination of cardiomyocyte apoptosis

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
arrhythmogenic right ventricular dysplasia 12		DOID:0110083	OMIM:611528	J:235770