Phenotypes associated with this allele

• Allele Symbol: Tg(H2-K-Fosl1)1Wag
• Allele Name: transgene insertion 1, Erwin F Wagner
• Allele ID: MGI:3639512
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Jund^tm1Mya/Jund^tm1Mya Tg(H2-K-Fosl1)1Wag/0	involves: 129S2/SvPas * C57BL/6 * CBA	MGI:3639603
tg2	Tg(H2-K-Fosl1)1Wag/0	involves: C57BL/6 * CBA	MGI:3639513

Genotype
MGI:3639603

cx1

• Allelic Composition: Jund^tm1Mya/Jund^tm1Mya; Tg(H2-K-Fosl1)1Wag/0
• Genetic Background: involves: 129S2/SvPas * C57BL/6 * CBA

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

mortality/aging

postnatal lethality, incomplete penetrance ( J:95691 )

• about 60% die within the first 2 weeks after birth

cardiovascular system

visceral vascular congestion ( J:95691 )

• show signs of advanced chronic hepatic and pulmonary congestion

liver vascular congestion ( J:95691 )

pulmonary vascular congestion ( J:95691 )

abnormal myocardial fiber morphology ( J:95691 )

• mitochondria are randomly dispersed in clusters and appear swollen

cardiac hypertrophy ( J:95691 )

• cardiomyocyte hypertrophy and disarray

cardiac interstitial fibrosis ( J:95691 )

• extensive focal interstitial fibrosis in ventricles

dilated cardiomyopathy ( J:95691 )

• heart size of adults is increased

congestive heart failure ( J:95691 )

cellular

cardiac interstitial fibrosis ( J:95691 )

• extensive focal interstitial fibrosis in ventricles

abnormal mitochondrial physiology ( J:95691 )

• MEFs exhibit a marked increase in basal polarization of mitochondria and fast mitochondrial depolarization in response to oligomycin

homeostasis/metabolism

edema ( J:95691 )

• mutants surviving the first month after birth develop peripheral edema

ascites ( J:95691 )

• mutants surviving the first month after birth develop ascites

behavior/neurological

lethargy ( J:95691 )

• mutants surviving the first month after birth become lethargic

muscle

abnormal myocardial fiber morphology ( J:95691 )

• mitochondria are randomly dispersed in clusters and appear swollen

dilated cardiomyopathy ( J:95691 )

• heart size of adults is increased

respiratory system

pulmonary vascular congestion ( J:95691 )

respiratory distress ( J:95691 )

• mutants surviving the first month after birth become dyspneic

liver/biliary system

liver vascular congestion ( J:95691 )

growth/size/body

cardiac hypertrophy ( J:95691 )

• cardiomyocyte hypertrophy and disarray

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
dilated cardiomyopathy		DOID:12930	OMIM:PS115200	J:95691

Genotype
MGI:3639513

tg2

• Allelic Composition: Tg(H2-K-Fosl1)1Wag/0
• Genetic Background: involves: C57BL/6 * CBA

mortality/aging

increased susceptibility to induced morbidity/mortality ( J:95691 )

• mortality is increased in mutants after aortic banding (3 of 8 died during follow-up)

premature death ( J:64494 )

• life span is up to 9 months of age

cellular

enhanced osteoblast differentiation ( J:64494 )

• increase in the number of active osteoblasts is due to accelerated differentiation of osteoprogenitors into mature osteoblasts rather than increased proliferation of osteoblasts

abnormal osteoblast physiology ( J:64494 )

• surfaces of bone trabeculae are nearly continuously covered by cuboidal osteoblasts and osteoid seams, indicating enlargement of the osteoblast compartment and functional activity

growth/size/body

postnatal growth retardation ( J:64494 )

• grow slower starting at 4 weeks of age

enlarged spleen ( J:64494 )

• show severe splenomegaly at around 9 months of age due to extramedullary hematopoiesis

skeleton

abnormal cranium morphology ( J:64494 )

• increase in radiodensity of the skull

abnormal neurocranium morphology ( J:64494 )

• calvariae display increased thickness and the bone marrow spaces are obliterated by bone tissue

thick neurocranium ( J:64494 )

abnormal long bone morphology ( J:64494 )

• increase in radiodensity of the distal and proximal ends of the long bones

• older mice display an increase in cortical thickness and in both the number and diameter of trabeculae in long bones

short femur ( J:64494 )

• femoral length is reduced at 8 weeks of age

abnormal long bone metaphysis morphology ( J:64494 )

• femoral metaphyses of 8 week old mutants show increased trabecular bone volume and osteoid volume and a 2-fold increase in osteoblast-covered bone surfaces

abnormal rib morphology ( J:64494 )

• increase in radiodensity of the ribs

kyphosis ( J:64494 )

• develop a hunched back with advancing age

abnormal vertebral body morphology ( J:64494 )

• increase in radiodensity of vertebral bodies

abnormal bone structure ( J:64494 )

enhanced osteoblast differentiation ( J:64494 )

• increase in the number of active osteoblasts is due to accelerated differentiation of osteoprogenitors into mature osteoblasts rather than increased proliferation of osteoblasts

increased compact bone thickness ( J:64494 )

• older mice display an increase in cortical thickness of long bones

increased bone mass ( J:64494 )

• show a dramatic postnatal increase in bone mass of the entire skeleton, first apparent at 4 weeks of age

osteosclerosis ( J:64494 )

• develop osteosclerosis as a result of increased skeletal mass due to increased bone formation

abnormal skeleton physiology ( J:64494 )

abnormal osteoblast physiology ( J:64494 )

• surfaces of bone trabeculae are nearly continuously covered by cuboidal osteoblasts and osteoid seams, indicating enlargement of the osteoblast compartment and functional activity

abnormal bone mineralization ( J:64494 )

• exhibit an increase in mineralizing bone surfaces and slightly elevated mineral apposition rates

• cultures of primary osteoblasts show higher amounts of mineralized matrix at days 16 and 18

hematopoietic system

enlarged spleen ( J:64494 )

• show severe splenomegaly at around 9 months of age due to extramedullary hematopoiesis

extramedullary hematopoiesis ( J:64494 )

anemia ( J:64494 )

• exhibit normochromic anemia at 3 months of age

immune system

enlarged spleen ( J:64494 )

• show severe splenomegaly at around 9 months of age due to extramedullary hematopoiesis

liver/biliary system

liver cirrhosis ( J:64494 )

• develops in some mutants

neoplasm

increased tumor incidence ( J:64494 )

• some mutants develop bronchoalveolar tumors but no bone tumors

limbs/digits/tail

short femur ( J:64494 )

• femoral length is reduced at 8 weeks of age

craniofacial

abnormal cranium morphology ( J:64494 )

• increase in radiodensity of the skull

abnormal neurocranium morphology ( J:64494 )

• calvariae display increased thickness and the bone marrow spaces are obliterated by bone tissue

thick neurocranium ( J:64494 )

cardiovascular system

decreased cardiac muscle contractility ( J:95691 )

• fractional shortening, velocity of circumferential shortening corrected for heart rate (VCFc) and peak aortic velocity corrected for heart rate (PAVc) are decreased, indicating reduced cardiac contractility

muscle

decreased cardiac muscle contractility ( J:95691 )

• fractional shortening, velocity of circumferential shortening corrected for heart rate (VCFc) and peak aortic velocity corrected for heart rate (PAVc) are decreased, indicating reduced cardiac contractility