Phenotypes associated with this allele
|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hbegftm1.1Mek mutation
(2 available);
any
Hbegf mutation
(25 available)
|
|
|
cardiovascular system
|
|
• atrioventricular valve thickness is increased compared to in wild-type mice
|
|
|
• outflow tract valve thickness is increased compared to in wild-type mice
|
|
|
• mesenchyme cells from cushion explants exhibit increased proliferation compared with similarly treated wild-type explants
• proliferation of outflow tract (OFT) and atrioventricular (AV) valve mesenchyme in culture is increased compared to in wild-type mice
• however, in vivo proliferation of endocardium, proliferation of endocardial cell from cushion explants, and apoptosis of OFT and AV endocardium and mesenchyme are normal
|
|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hbegftm1.1Mek mutation
(2 available);
any
Hbegf mutation
(25 available)
|
|
|
mortality/aging
|
|
• more than 50% of homozygotes that survive the first postnatal week die at P25
|
|
|
• over 60% of homozygotes die within the first week of life
|
cardiovascular system
|
|
• at 12 weeks, the size of mutant cardiomyocytes is enlarged by ~2-fold relative to that in control mice, indicating myofiber hypertrophy
• however, no cardiomyocyte hypertrophy is noted at E19.5
|
|
|
• at E19.5, mutant hearts exhibit enlarged atrioventricular valves with an abnormal thickened globular morphology relative to control hearts
• however, no fibrosis is observed, suggesting that valve thickening is due to an increased number of mesenchymal cells
|
|
|
• mutant mitral valves appear thickened both at E19.5 and at 12 weeks of age
• at 12 weeks, the rate of thickening of the mitral valve is relatively lower than that of the aortic valve
|
|
|
• at E19.5, mutant tricuspid valves appear thickened relative to control valves
|
|
|
• mutant mitral valves appear enlarged and thickened both at E19.5 and at 12 weeks of age
|
|
|
• at E19.5, mutant tricuspid valves appear enlarged relative to control valves
|
|
|
• at 6 weeks of age, homozygotes display massively enlarged hearts
• heart enlargement is evident as early as E19.5
|
|
|
• at E19.5, mutant hearts exhibit enlarged semilunar valves with an abnormal thickened globular morphology relative to control hearts
• however, no fibrosis is observed, suggesting that valve thickening is due to an increased number of mesenchymal cells
|
|
|
• mutant aortic valves appear thickened both at E19.5 and at 12 weeks of age
|
|
|
• at E19.5, mutant pulmonary valves appear thickened relative to control valves
|
|
|
• mutant aortic valves appear enlarged and thickened both at E19.5 and at 12 weeks of age
|
|
|
• at E19.5, mutant pulmonary valves appear enlarged relative to control valves
|
|
|
• at E19.5, both left (LV) and right (RV) ventricular chambers are dilated relative to those in control hearts
• at 12 weeks of age, homozygotes show progressive dilation of both ventricular chambers relative to controls
|
|
|
• at 8-12 weeks, transthoracic echocardiography indicates marked dilation of the LV diameter, with an average LV end-diastolic value of 4.53 mm vs 2.87 mm in control mice
|
|
|
• at 8-12 weeks, the ventricular fractional shortening (FS) is reduced to 29% in mutant mice relative to 49% in control mice
• however, no differences in body weight, heart rate, and systolic or diastolic blood pressures are observed relative to control mice
|
muscle
|
|
• at 12 weeks, the size of mutant cardiomyocytes is enlarged by ~2-fold relative to that in control mice, indicating myofiber hypertrophy
• however, no cardiomyocyte hypertrophy is noted at E19.5
|
|
|
• at 8-12 weeks, the ventricular fractional shortening (FS) is reduced to 29% in mutant mice relative to 49% in control mice
• however, no differences in body weight, heart rate, and systolic or diastolic blood pressures are observed relative to control mice
|
growth/size/body
|
|
• at 6 weeks of age, homozygotes display massively enlarged hearts
• heart enlargement is evident as early as E19.5
|
|
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hbegftm1.1Mek mutation
(2 available);
any
Hbegf mutation
(25 available)
|
|
|
cardiovascular system
|
|
• mesenchyme cells from cushion explants exhibit increased proliferation compared with similarly treated wild-type explants
• however, proliferation of endocardial cells from cushion explants is normal
|
Allelic
Composition |
Hbegftm1.1Mek/Hbegf+
|
|
Genetic
Background |
involves: C57BL/6 * C57BL/6J * CBA |
|
| Find Mice |
Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hbegftm1.1Mek mutation
(2 available);
any
Hbegf mutation
(25 available)
|
|
|
normal phenotype
|
|
• heterozygotes display no overt abnormalities
|
Analysis Tools