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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Hey2tm1Gess
targeted mutation 1, Manfred Gessler
MGI:2387937
Summary 7 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
hm1
Hey2tm1Gess/Hey2tm1Gess B6.129P2-Hey2tm1Gess MGI:3620644
hm2
Hey2tm1Gess/Hey2tm1Gess involves: 129P2/OlaHsd MGI:3789050
hm3
Hey2tm1Gess/Hey2tm1Gess involves: 129P2/OlaHsd * C57BL/6 MGI:2450864
hm4
Hey2tm1Gess/Hey2tm1Gess involves: 129P2/OlaHsd * CD-1 MGI:3620645
cx5
Hey1tm1Gess/Hey1+
Hey2tm1Gess/Hey2tm1Gess
involves: 129 * C57BL/6 MGI:3043227
cx6
Hey1tm1Gess/Hey1tm1Gess
Hey2tm1Gess/Hey2+
involves: 129 * C57BL/6 MGI:3043228
cx7
Hey1tm1Gess/Hey1tm1Gess
Hey2tm1Gess/Hey2tm1Gess
involves: 129 * C57BL/6 MGI:3043229


Genotype
MGI:3620644
hm1
Allelic
Composition
Hey2tm1Gess/Hey2tm1Gess
Genetic
Background
B6.129P2-Hey2tm1Gess
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hey2tm1Gess mutation (0 available); any Hey2 mutation (30 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Ventricular and atrial septal defects in Hey2tm1Gess/Hey2tm1Gess mice

mortality/aging
• Background Sensitivity: 80-90% die within 10 days after birth, unlike on a CD-1 background in which lethality rates are much lower

cardiovascular system
• Background Sensitivity: exhibit more severe cardiac defects on the C57BL/6 background than on a CD-1 background
• 8 of 15 at E13.5-E16.5 lack an open connection between the right atrium and ventricle, although the leaflets of the tricuspid valve are formed
• show persistent foramen ovale
• Background Sensitivity: ventricular septal defects are much more severe than on a CD-1 background
• high incidence of ventricular septal defects that are consistently located in the upper membranous part of the ventricular septum, just below the atrioventricular junction
• the few mice surviving to adulthood show altered heart shapes but no valve or septum defects




Genotype
MGI:3789050
hm2
Allelic
Composition
Hey2tm1Gess/Hey2tm1Gess
Genetic
Background
involves: 129P2/OlaHsd
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hey2tm1Gess mutation (0 available); any Hey2 mutation (30 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• atrioventricular canal explants from E9.5 embryos show a strong reduction in the number of fully transformed, elongated mesenchymal cells indicating impaired epithelial to mesenchymal transition
• at E15.5, all mice show a ventricular membranous septal defect very similar to that in Heyltm1Gess Hey1tm1Gess double homozygous mice




Genotype
MGI:2450864
hm3
Allelic
Composition
Hey2tm1Gess/Hey2tm1Gess
Genetic
Background
involves: 129P2/OlaHsd * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hey2tm1Gess mutation (0 available); any Hey2 mutation (30 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• incomplete penetrance; 80% fail to thrive and die by 10 days of age

cardiovascular system
• fiber disarray
• only identified in higher C57BL/6 backcross generations
• only identified in higher C57BL/6 backcross generations
• 30-300% increase in heart to body weight ratios
• normal aorta, however, with normal diameter and no evidence of stenosis or atresia
• mild fibrosis

growth/size/body
• 30-300% increase in heart to body weight ratios
• normal aorta, however, with normal diameter and no evidence of stenosis or atresia
• failure to thrive within the first 10 days of life
• progeny that survive to weaning gradually reach normal body weight by 2-3 mo

muscle
• fiber disarray




Genotype
MGI:3620645
hm4
Allelic
Composition
Hey2tm1Gess/Hey2tm1Gess
Genetic
Background
involves: 129P2/OlaHsd * CD-1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hey2tm1Gess mutation (0 available); any Hey2 mutation (30 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Ventricular and atrial septal defects in Hey2tm1Gess/Hey2tm1Gess mice

mortality/aging
• Background Sensitivity: lethality rates are much lower than in the C57BL/6 background, and in higher backcross generations almost all homozygotes survive

cardiovascular system
• Background Sensitivity: 55.6% of embryos at E16.5 show ventricular septal defects, much less than on a CD-1 background, and do not show any other cardiac defects




Genotype
MGI:3043227
cx5
Allelic
Composition
Hey1tm1Gess/Hey1+
Hey2tm1Gess/Hey2tm1Gess
Genetic
Background
involves: 129 * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hey1tm1Gess mutation (0 available); any Hey1 mutation (17 available)
Hey2tm1Gess mutation (0 available); any Hey2 mutation (30 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• by P21 - P28 far fewer mutants are found than expected
• adult mutants also display additional adult lethality from an unknown caus




Genotype
MGI:3043228
cx6
Allelic
Composition
Hey1tm1Gess/Hey1tm1Gess
Hey2tm1Gess/Hey2+
Genetic
Background
involves: 129 * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hey1tm1Gess mutation (0 available); any Hey1 mutation (17 available)
Hey2tm1Gess mutation (0 available); any Hey2 mutation (30 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• embryonic survival is reduced by 60% compared to wild-type of Hey1tm1Gess homozygous littermates




Genotype
MGI:3043229
cx7
Allelic
Composition
Hey1tm1Gess/Hey1tm1Gess
Hey2tm1Gess/Hey2tm1Gess
Genetic
Background
involves: 129 * C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Hey1tm1Gess mutation (0 available); any Hey1 mutation (17 available)
Hey2tm1Gess mutation (0 available); any Hey2 mutation (30 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• no double homozygous embryos survive and all show abnormalities by E10.5

cardiovascular system
• reduction in expression of arterial specific markers suggests impaired arterial cell fate specification in double homozygotes
• the dorsal aortae are frequently reduced or missing on one or both sides of double homozygous embryos probably as a result of impaired aortic wall formation
• intersomitic vessels formed by angiogenetic sprouting do form but appear less organized than in controls
• at E10.5 no embryonic blood vessels can be detected in the labyrinthine layer
• however, embryonic vessels are seen in the allantois and chorionic plate
• the cardinal veins are frequently reduced or missing on one or both sides of double homozygous embryos
• vascular patterning is coarse especially in the head with many truncated vessels without finely branched trees
• at E9.5 the heart lacks trabeculae
• at E9.5 the heart has initiated looping but the myocardium is much thinner
• at E9.5 - E10.5 the ventricular portion of the heart does not fully enlarge however contractions and blood circulation appear to occur
• at E10.5 balloon like pericardial sacs are seen in homozygous mutants
• at E9.5 evidence of hemorrhage can be seen
• at E10.5 massive hemorrhage is seen in the head, trunk, and pericardial cavity of homozygous mutants

embryo
• at E9.5 double homozygous embryos appear developmentally retarded
• at E9.5 the neural tube is thinner and the surrounding mesenchymal cells appear sparse
• somite formation ceases after E9.5 after 21-26 somites although the somites that do form are normal in size and shape
• at E10.5 the labyrinthine layer lacks fetal blood circulation and appears very cell-rich without the intermingling of maternal and embryonic blood spaces seen in controls
• at E10.5 no embryonic blood vessels can be detected in the labyrinthine layer
• however, embryonic vessels are seen in the allantois and chorionic plate
• formation of an umbilical cord is initiated; however, after E9.5 the connection between embryo and placenta does not mature into a thicker umbilical cord
• at E9.5 the primitive vascular plexus is present but fails to remodel and by E10.5 is unchanged or degenerating
• by E11.5 when most double homozygous embryos are necrotic no vasculature is left in the yolk sac

muscle
• at E9.5 the heart lacks trabeculae
• at E9.5 the heart has initiated looping but the myocardium is much thinner

nervous system
• at E9.5 the neural tube is thinner and the surrounding mesenchymal cells appear sparse

growth/size/body
• at E9.5 double homozygous embryos appear developmentally retarded





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last database update
04/23/2024
MGI 6.23
The Jackson Laboratory