Phenotypes Associated with This Genotype

Genotype
MGI:4867270

• Allelic Composition: Lims1^tm1.1Chen/Lims1^tm1.1Chen; Lims2^tm1.1Chen/Lims2^tm1.1Chen; Tg(Tnnt2-cre)5Blh/0
• Genetic Background: involves: 129/Sv * C57BL/6 * DBA/2

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

mortality/aging

premature death ( J:167114 )

• mice die within 4 weeks

postnatal lethality, incomplete penetrance ( J:167114 )

• mice die within 4 weeks

cardiovascular system

liver vascular congestion ( J:167114 )

abnormal pectinate muscle morphology ( J:167114 )

• mice show abnormalities in pectinate muscles in atrial appendages

decreased myocardial fiber size ( J:167114 )

• cultured attached cardiomyocytes are smaller than similarly treated wild-type cells

thin myocardium compact layer ( J:167114 )

• significantly thinner compact myocardium at P1

abnormal ventricle myocardium morphology ( J:167114 )

• at P1, myocardium exhibit widened gaps of intercalated disks with disarrayed sarcomeres and distorted Z lines compared to in wild-type mice

• at P10, membranes of the intercalated disk are highly convoluted and gaps are widened unlike in wild-type mice

• the myocardium exhibits disruption of cell-cell adhesion compared to in wild-type mice

dilated heart atrium ( J:167114 )

• at P20

abnormal heart shape ( J:167114 )

• hearts are abnormally shaped with a bulge at the base of the right ventricle at P20

globular heart ( J:167114 )

• hearts are rounder at P1

abnormal heart left ventricle morphology ( J:167114 )

• mice exhibit disorganized trabeculae that fail to fuse and incorporate into compact myocardium unlike in wild-type mice

dilated heart left ventricle ( J:167114 )

• at P1 and P20

abnormal heart ventricle wall thickness ( J:167114 )

• at P20, the ventricular wall exhibits irregular thickness with a thickened right ventricular free wall and thin left ventricular free wall and septum compared to in wild-type mice

cardiac fibrosis ( J:167114 )

• at P20, hearts show fibrosis, esp. in the ventricular septum and within thinned regions of ventricular myocardium

abnormal myocardial fiber physiology ( J:167114 )

• few cardiomyocytes attack to a plate coated in fibronectin, laminin, and collagen compared with wild-type cells

congestive heart failure ( J:167114 )

• progressive, resulting in death

homeostasis/metabolism

abnormal atrial thrombosis ( J:167114 )

• at P20, a large intracardiac thrombus is frequently detected in the left atria

edema ( J:167114 )

• extensive in the lungs and liver

pulmonary edema ( J:167114 )

ascites ( J:167114 )

liver/biliary system

liver vascular congestion ( J:167114 )

enlarged liver ( J:167114 )

respiratory system

increased lung weight ( J:167114 )

pulmonary edema ( J:167114 )

growth/size/body

postnatal growth retardation ( J:167114 )

• gradual

increased lung weight ( J:167114 )

enlarged liver ( J:167114 )

muscle

abnormal pectinate muscle morphology ( J:167114 )

• mice show abnormalities in pectinate muscles in atrial appendages

decreased myocardial fiber size ( J:167114 )

• cultured attached cardiomyocytes are smaller than similarly treated wild-type cells

thin myocardium compact layer ( J:167114 )

• significantly thinner compact myocardium at P1

abnormal ventricle myocardium morphology ( J:167114 )

• at P1, myocardium exhibit widened gaps of intercalated disks with disarrayed sarcomeres and distorted Z lines compared to in wild-type mice

• at P10, membranes of the intercalated disk are highly convoluted and gaps are widened unlike in wild-type mice

• the myocardium exhibits disruption of cell-cell adhesion compared to in wild-type mice