Phenotypes Associated with This Genotype

Genotype
MGI:3043007

• Allelic Composition: Myl2^tm1(cre)Krc/Myl2⁺; Nkx2-5^tm1Krc/Nkx2-5^tm1Krc
• Genetic Background: either: (involves: 129/Sv * Black Swiss) or (involves: 129/Sv * C57BL/6)

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

mortality/aging

perinatal lethality, incomplete penetrance ( J:89404 )

• incomplete penetrance; mice were born at 78% of the expected rate

• pernatal lethality was reported to be more penetrant with contributions from the C57BL/6 genetic background

cardiovascular system

cardiovascular system phenotype ( J:89404 )

N • no atrial or ventricular septal defects

abnormal impulse conducting system morphology ( J:89404 )

abnormal atrioventricular bundle morphology ( J:89404 )

• bundle of His was hypoplastic at birth

atrioventricular node hypoplasia ( J:89404 )

• atrioventricular (AV) node was hypoplastic at birth

abnormal myocardial fiber morphology ( J:89404 )

• loss of integrity and sarcomeric structure in conduction system cardiomyocytes

• surrounding, non-conducting, cardiomyoctes were unaffected

abnormal myocardial trabeculae morphology ( J:89404 )

• hypertrabeculation; trabecular muscle overgrowth, filling the left ventricle

cardiac hypertrophy ( J:89404 )

cardiac interstitial fibrosis ( J:89404 )

dilated cardiomyopathy ( J:89404 )

atrioventricular block ( J:89404 )

• progressive AV block associated with cardiomyocyte dropout and fibrosis in the central conduction system

homeostasis/metabolism

hydrops fetalis ( J:89404 )

• fetuses that died prior to birth were edematous, suggesting circulatory failure

muscle

abnormal impulse conducting system morphology ( J:89404 )

abnormal atrioventricular bundle morphology ( J:89404 )

• bundle of His was hypoplastic at birth

atrioventricular node hypoplasia ( J:89404 )

• atrioventricular (AV) node was hypoplastic at birth

abnormal myocardial fiber morphology ( J:89404 )

• loss of integrity and sarcomeric structure in conduction system cardiomyocytes

• surrounding, non-conducting, cardiomyoctes were unaffected

abnormal myocardial trabeculae morphology ( J:89404 )

• hypertrabeculation; trabecular muscle overgrowth, filling the left ventricle

dilated cardiomyopathy ( J:89404 )

growth/size/body

cardiac hypertrophy ( J:89404 )

cellular

cardiac interstitial fibrosis ( J:89404 )