All Phenotypes Zfpm2<tm1Sho> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Zfpm2^tm1Sho
targeted mutation 1, Stuart Orkin
MGI:2386183

Summary

12 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Zfpm2^tm1Sho/Zfpm2^tm1Sho	involves: 129S1/Sv	MGI:2665413
ht2	Zfpm2^tm1Sho/Zfpm2⁺	C57BL/6JEi-Chr Y^A/HeJ/EiJ	MGI:6479109
ht3	Zfpm2^lil/Zfpm2^tm1Sho	involves: 129S1/Sv * A/J	MGI:3589478
cn4	Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Wap-cre)11738Mam/0	involves: 129 * C57BL/6J * C57BL/6NCr	MGI:3709976
cn5	Wt1^{tm1(EGFP/cre)Wtp}/Wt1⁺ Zfpm2^tm1Sho/Zfpm2^tm2Sho	involves: 129S1/Sv * 129S4/SvJae * 129S7/SvEvBrd	MGI:3851402
cn6	Gt(ROSA)26Sor^tm1Sho/Gt(ROSA)26Sor⁺ Wt1^{tm1(EGFP/cre)Wtp}/Wt1⁺ Zfpm2^tm1Sho/Zfpm2^tm1Sho	involves: 129S1/Sv * 129S4/SvJaeSor	MGI:3851406
cn7	Zfpm2^tm1Sho/Zfpm2^tm2Sho Nkx2-5^tm1(cre)Rjs/Nkx2-5⁺	involves: 129S1/Sv * 129S7/SvEvBrd	MGI:3851399
cn8	Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Tnnt2-rtTA,tetO-cre)1Wtp/0	involves: 129S1/Sv * 129S7/SvEvBrd	MGI:3851407
cn9	Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Myh6-cre)2182Mds/0	involves: 129S1/Sv * 129S7/SvEvBrd	MGI:3851401
cn10	Zfpm2^tm1Sho/Zfpm2^tm2Sho H2az2^{Tg(Wnt1-cre)11Rth}/H2az2⁺	involves: 129S1/Sv * C57BL/6 * CBA	MGI:3851405
cn11	Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Tek-cre)1Ywa/0	involves: 129S1/Sv * C57BL/6 * SJL	MGI:3851403
cx12	Zfpm2^tm1Sho/Zfpm2^tm1Sho Tg(Myh6-Zfpm2)1Sho/0	involves: 129S1/Sv	MGI:2665480

Allelic Composition	Zfpm2^tm1Sho/Zfpm2^tm1Sho
Genetic Background	involves: 129S1/Sv

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)

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

Abnormal lung development in the Zfpm2^tm1Sho/Zfpm2^tm1Sho mouse

muscle

thin ventricle myocardium compact layer ( J:62879 )

• at E13.5-E15.0, mutant embryos display thinning of the compact ventricular myocardium, similar to a "papyraceous phenotype"

ventricular myocardium compact layer hypoplasia ( J:62879 )

• a striking ventricular compact layer hypoplasia is observed

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:62879 )

• mutant embryos die at midgestation between E12.5 and E15.5; some are found dead by E13.5

• at E14.5, approximately 50% of mutant embryos are necrotic and undergoing resorption

cardiovascular system

supravalvar pulmonary trunk stenosis ( J:62879 )

• a severely obstructed pulmonary trunk (stenosis) is observed at E12.5

thin ventricle myocardium compact layer ( J:62879 )

• at E13.5-E15.0, mutant embryos display thinning of the compact ventricular myocardium, similar to a "papyraceous phenotype"

ventricular myocardium compact layer hypoplasia ( J:62879 )

• a striking ventricular compact layer hypoplasia is observed

abnormal cardiac epithelial to mesenchymal transition ( J:62879 )

• an early developmental block prevents epithelial-to-mesenchymal transition of "transforming" epicardial cells and subsequent formation of coronary vascular plexus within the subepicardial space

absent coronary vessels ( J:62879 )

• coronary vasculature is absent, despite formation of an intact epicardial layer and expression of epicardium-specific genes

overriding aortic valve ( J:62879 )

• an overriding aorta is detected at E13.5-E15.0

common atrioventricular valve ( J:62879 )

• a common atrioventricular valve is situated between the left and right ventricles

abnormal heart atrium morphology ( J:62879 )

• at E13.5, mutant atria are thinner than normal and only a few trabeculae are observed

atrial septal defect ( J:62879 )

• atrial septal defects are detected at E13.5-E15.5

dilated heart atrium ( J:62879 )

• at E13.5, mutant atria are significantly dilated

ventricular septal defect ( J:62879 )

• a subaortic ventricular septal defect is noted at E13.5-E15.0, as part of a complete common atrioventricular canal

globular heart ( J:62879 )

• by 13.5, mutant hearts are rounder than normal, as sharp ventricular apicies are absent

heart right ventricle outflow tract stenosis ( J:62879 )

• subpulmonic stenosis is detected at E13.5-E15.0

thin ventricular wall ( J:62879 )

hemorrhage ( J:62879 )

• at E13.5, mutant embryos often display peripheral hemorrhage

• however, no bleeding is noted around the heart

decreased heart rate ( J:62879 )

• by E13.5, pulsation is weaker and slower, and blood cells remain within the heart even after contraction

congestive heart failure ( J:62879 )

• at E13.5, mutant embryos display an overall appearance consistent with heart failure

respiratory system

abnormal lung development ( J:62879 , J:100119 )

• underdevelopment of lungs is observed at E13.5-E15, secondary to heart failure (J:62879)

• branching in unaffected lobes appears delayed by 6 - 12 hours in homozygous lung culture explants; however, all mutant lungs do develop the normal branching pattern after 5 days in culture (J:100119)

absent right lung accessory lobe ( J:100119 )

• the right lung accessory lobe fails to develop

pulmonary hypoplasia ( J:62879 , J:100119 )

• at E12, prior to onset of diaphragm function, mutant lungs are smaller and lack development of an accessory lobe (J:100119)

homeostasis/metabolism

hydrops fetalis ( J:62879 )

• at E13.5, mutant embryos are edematous

liver/biliary system

small liver ( J:62879 )

• a smaller liver size is observed at E13.5-E15.5, secondary to heart failure

integument

pallor ( J:62879 )

• at E13.5, mutant embryos are pale

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
tetralogy of Fallot		DOID:6419	OMIM:187500	J:62879 , J:78688

Allelic Composition	Zfpm2^tm1Sho/Zfpm2⁺
Genetic Background	C57BL/6JEi-Chr Y^A/HeJ/EiJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)

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

reproductive system

ovotestis ( J:125196 )

primary sex reversal ( J:125196 )

• Background Sensitivity: heterozygous carriers with the A/HeJ Y Chromosome on the C57BL/6JEi background develop ovaries and ovotestes

true hermaphroditism ( J:125196 )

Allelic Composition	Zfpm2^lil/Zfpm2^tm1Sho
Genetic Background	involves: 129S1/Sv * A/J

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Zfpm2^lil mutation (0 available); any Zfpm2 mutation (47 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)

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

mortality/aging

prenatal lethality, incomplete penetrance ( J:100119 )

• less than 5% of expected numbers of mutants survive to birth

Allelic Composition	Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Wap-cre)11738Mam/0
Genetic Background	involves: 129 * C57BL/6J * C57BL/6NCr

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Wap-cre)11738Mam mutation (3 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)
Zfpm2^tm2Sho mutation (1 available); any Zfpm2 mutation (47 available)

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

reproductive system

reproductive system phenotype ( J:120985 )

N	• mice are fertile

endocrine/exocrine glands

abnormal mammary gland morphology ( J:120985 )

• at day 4 of involution, alveoli are completely collapsed into clusters of epithelial cells and the area containing adipocytes is greatly expanded

• however, after 6 days of involution there is no difference when compared to controls

abnormal involution of the mammary gland ( J:120985 )

• involution occurs faster at days 3 and 4 of involution than in controls

integument

abnormal mammary gland morphology ( J:120985 )

• at day 4 of involution, alveoli are completely collapsed into clusters of epithelial cells and the area containing adipocytes is greatly expanded

• however, after 6 days of involution there is no difference when compared to controls

abnormal involution of the mammary gland ( J:120985 )

• involution occurs faster at days 3 and 4 of involution than in controls

Allelic Composition	Wt1^{tm1(EGFP/cre)Wtp}/Wt1⁺ Zfpm2^tm1Sho/Zfpm2^tm2Sho
Genetic Background	involves: 129S1/Sv * 129S4/SvJae * 129S7/SvEvBrd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Wt1^{tm1(EGFP/cre)Wtp} mutation (1 available); any Wt1 mutation (34 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)
Zfpm2^tm2Sho mutation (1 available); any Zfpm2 mutation (47 available)

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

mortality/aging

perinatal lethality, complete penetrance ( J:150452 )

• pups die in perinatal period

cardiovascular system

cardiovascular system phenotype ( J:150452 )

N	• coronary vascular development is not impaired

thin myocardium compact layer ( J:150452 )

• compact myocardium is thin

abnormal atrioventricular cushion morphology ( J:150452 )

• severe atrio-ventricular endocardial cushion defect is observed by E14.5

muscle

thin myocardium compact layer ( J:150452 )

• compact myocardium is thin

Allelic Composition	Gt(ROSA)26Sor^tm1Sho/Gt(ROSA)26Sor⁺ Wt1^{tm1(EGFP/cre)Wtp}/Wt1⁺ Zfpm2^tm1Sho/Zfpm2^tm1Sho
Genetic Background	involves: 129S1/Sv * 129S4/SvJaeSor

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^tm1Sho mutation (4 available); any Gt(ROSA)26Sor mutation (967 available)
Wt1^{tm1(EGFP/cre)Wtp} mutation (1 available); any Wt1 mutation (34 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)

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

cardiovascular system

cardiovascular system phenotype ( J:150452 )

N	• mice show normal numbers of epicardium-derived cells (EPDCs) and epicardial epithelial-mesenchymal transition (EMT) occurs normally

Allelic Composition	Zfpm2^tm1Sho/Zfpm2^tm2Sho Nkx2-5^tm1(cre)Rjs/Nkx2-5⁺
Genetic Background	involves: 129S1/Sv * 129S7/SvEvBrd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Nkx2-5^tm1(cre)Rjs mutation (1 available); any Nkx2-5 mutation (21 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)
Zfpm2^tm2Sho mutation (1 available); any Zfpm2 mutation (47 available)

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

Abnormal heart development and coronary vasculogenesis in Nkx2-5^tm1(cre)Rjs/Nkx2-5⁺ Zfpm2^tm1Sho/Zfpm2^tm2Sho mice

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:150452 )

• embryos die by E13.5-E14.5

cardiovascular system

thin myocardium compact layer ( J:150452 )

• compact myocardium is thin

abnormal atrioventricular cushion morphology ( J:150452 )

• atrio-ventricular cushion defect is observed

abnormal coronary vessel morphology ( J:150452 )

• coronary vascular plexus is significantly decreased compared to controls; myocardium contains few coronary vessels

overriding aortic valve ( J:150452 )

atrial septal defect ( J:150452 )

• large atrial septal defect is observed in embryos

ventricular septal defect ( J:150452 )

• large ventricular septal defect is observed

pericardial effusion ( J:150452 )

• embryos display pericardial effusion prior to death

hemorrhage ( J:150452 )

• embryos display hemorrhage prior to death

homeostasis/metabolism

pericardial effusion ( J:150452 )

• embryos display pericardial effusion prior to death

skin edema ( J:150452 )

• embryos develop subcutaneous edema prior to death

integument

skin edema ( J:150452 )

• embryos develop subcutaneous edema prior to death

muscle

thin myocardium compact layer ( J:150452 )

• compact myocardium is thin

Allelic Composition	Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Tnnt2-rtTA,tetO-cre)1Wtp/0
Genetic Background	involves: 129S1/Sv * 129S7/SvEvBrd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Tnnt2-rtTA,tetO-cre)1Wtp mutation (0 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)
Zfpm2^tm2Sho mutation (1 available); any Zfpm2 mutation (47 available)

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

Increased fibrotic tissues in doxycycline-treated Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Tnnt2-rtTA,tetO-cre)1Wtp/0 hearts

cardiovascular system

abnormal cardiovascular development ( J:150452 )

• decreased coronary vasculature is observed in doxycycline-treated mice relative to controls

decreased cardiac muscle contractility ( J:150452 )

• significantly diminished fractional shortening is observed in mice receiving doxycycline-treated water from 4 to 8 weeks of age

• fractional shortening progressively decreases in animals treated with doxycycline

increased cardiomyocyte apoptosis ( J:150452 )

• level of apoptosis is elevated compared to controls

muscle

decreased cardiac muscle contractility ( J:150452 )

• significantly diminished fractional shortening is observed in mice receiving doxycycline-treated water from 4 to 8 weeks of age

• fractional shortening progressively decreases in animals treated with doxycycline

increased cardiomyocyte apoptosis ( J:150452 )

• level of apoptosis is elevated compared to controls

cellular

increased cardiomyocyte apoptosis ( J:150452 )

• level of apoptosis is elevated compared to controls

Allelic Composition	Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Myh6-cre)2182Mds/0
Genetic Background	involves: 129S1/Sv * 129S7/SvEvBrd

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-cre)2182Mds mutation (3 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)
Zfpm2^tm2Sho mutation (1 available); any Zfpm2 mutation (47 available)

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

Dilated cardiomyopathy in Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Myh6-cre)2182Mds/0 mice

mortality/aging

premature death ( J:150452 )

• mice survive normally to weaning but die at 8-12 weeks

postnatal lethality, complete penetrance ( J:150452 )

cardiovascular system

cardiovascular system phenotype ( J:150452 )

N	• at E14.5 no structural heart defects or coronary vascular plexus abnormalities are observed • myocardium thickens normally and contains normal number of intramyocardial vessels

abnormal cardiovascular development ( J:150452 )

• decreased coronary vasculature is observed in adults

• density of PECAM positive vessels particularly capillaries are reduced in density; coronary arteriole density is also decreased

cardiac fibrosis ( J:150452 )

• significantly increased and replaces apoptotic tissues

dilated heart ( J:150452 )

• adult hearts are dilated

dilated heart ventricle ( J:150452 )

• ventricular dilation is observed

abnormal coronary circulation ( J:150452 )

• decreased myocardial perfusion is observed in adults, resulting in tissue hypoxia

decreased cardiac muscle contractility ( J:150452 )

• fractional shortening is severely depressed in adults

decreased heart ventricle muscle contractility ( J:150452 )

• contraction is depressed in adults

increased cardiomyocyte apoptosis ( J:150452 )

• significantly increased in adult heart compared to controls

muscle

decreased cardiac muscle contractility ( J:150452 )

• fractional shortening is severely depressed in adults

decreased heart ventricle muscle contractility ( J:150452 )

• contraction is depressed in adults

increased cardiomyocyte apoptosis ( J:150452 )

• significantly increased in adult heart compared to controls

cellular

increased cardiomyocyte apoptosis ( J:150452 )

• significantly increased in adult heart compared to controls

Allelic Composition	Zfpm2^tm1Sho/Zfpm2^tm2Sho H2az2^{Tg(Wnt1-cre)11Rth}/H2az2⁺
Genetic Background	involves: 129S1/Sv * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
H2az2^{Tg(Wnt1-cre)11Rth} mutation (2 available); any H2az2 mutation (26 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)
Zfpm2^tm2Sho mutation (1 available); any Zfpm2 mutation (47 available)

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

mortality/aging

mortality/aging ( J:150452 )

N	• mice survive normally

cardiovascular system

cardiovascular system phenotype ( J:150452 )

N	• no detectable defects in heart morphogenesis or coronary development are detected

Allelic Composition	Zfpm2^tm1Sho/Zfpm2^tm2Sho Tg(Tek-cre)1Ywa/0
Genetic Background	involves: 129S1/Sv * C57BL/6 * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Tek-cre)1Ywa mutation (6 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)
Zfpm2^tm2Sho mutation (1 available); any Zfpm2 mutation (47 available)

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

mortality/aging

mortality/aging ( J:150452 )

N	• survival rate to weaning is normal

cardiovascular system

cardiovascular system phenotype ( J:150452 )

N	• coronary plexus, endocardial cushions and compact myocardial layer develop normally • adults have normal tricuspid and mitral valves

Allelic Composition	Zfpm2^tm1Sho/Zfpm2^tm1Sho Tg(Myh6-Zfpm2)1Sho/0
Genetic Background	involves: 129S1/Sv

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(Myh6-Zfpm2)1Sho mutation (1 available)
Zfpm2^tm1Sho mutation (2 available); any Zfpm2 mutation (47 available)

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

mortality/aging

lethality throughout fetal growth and development, complete penetrance ( J:62879 )

• fetuses survive to about E17.5

cardiovascular system

cardiovascular system phenotype ( J:62879 )

N	• at E15.5, mutant embryos are grossly normal and display normal coronary vasculogenesis • no ventricular wall thinning is observed

common atrioventricular valve ( J:62879 )

• a common atrioventricular valve is situated between the left and right ventricles

endocrine/exocrine glands

abnormal ovary morphology ( J:78688 )

abnormal Sertoli cell morphology ( J:78688 )

• blocked differentiation

abnormal Leydig cell differentiation ( J:78688 )

• maturation of Leydig cells was not initiated

reproductive system

abnormal ovary morphology ( J:78688 )

abnormal Sertoli cell morphology ( J:78688 )

• blocked differentiation

abnormal Leydig cell differentiation ( J:78688 )

• maturation of Leydig cells was not initiated

abnormal primary sex determination ( J:78688 )

• XX mice have abnormal ovaries; details not given

• gonads of XY mice are abnormal

primary sex reversal ( J:78688 )

• gonads have ovarian shape and location at E17.5, germ cells and vasculature present but no testicular cords

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