Phenotypes associated with this allele

• Allele Symbol: Tg(AGER)#Eca
• Allele Name: transgene insertion, Eleonora Cavarra
• Allele ID: MGI:5569820
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
tg1	Tg(AGER)#Eca/Tg(AGER)#Eca	C57BL/6J-Tg(AGER)#Eca	MGI:5569826
tg2	Tg(AGER)#Eca/0	C57BL/6J-Tg(AGER)#Eca	MGI:5569821

Genotype
MGI:5569826

tg1

• Allelic Composition: Tg(AGER)#Eca/Tg(AGER)#Eca
• Genetic Background: C57BL/6J-Tg(AGER)#Eca

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

mortality/aging

postnatal lethality, incomplete penetrance ( J:205092 )

• postnatal mortality greater than 50% due to respiratory failure

growth/size/body

postnatal growth retardation ( J:205092 )

• delay in body development

respiratory system

abnormal lung vasculature morphology ( J:205092 )

• abnormal vascular development

• vascular changes consist of mild medial thickening and abnormalities in elastic fiber architecture

decreased lung endothelial cell proliferation ( J:205092 )

• cell proliferation is reduced in lung epithelia at the beginning of the alveolarization phase compared to controls which show an increase in proliferation

abnormal lung alveolus development ( J:205092 )

• poor alveolar development, with an increase in mean linear intercept and a decrease in internal surface area of lungs

• decrease in volume density ratio of secondary alveolar crests

abnormal lung interstitium morphology ( J:205092 )

• areas of alveolar interstitium thickening

abnormal pulmonary alveolar system morphology ( J:205092 )

• alveolar hypoplasia, with a decrease in alveolar number and internal surface area

abnormal pulmonary alveolus wall morphology ( J:205092 )

• thin alveolar walls

• mice show an increase in the number of TUNEL-positive cells in the alveolar walls at P4, P8, P20 and P30 indicating increased apoptosis

overexpanded pulmonary alveolus ( J:205092 )

• dilated airspaces, with thin alveolar walls interspersed between focal and widened septa

abnormal pulmonary interalveolar septum morphology ( J:205092 )

• thin alveolar walls are interspersed between focal and widened septa

• hypercellular septa

emphysema ( J:205092 )

• increase in alveolar destructive index and persistent inflammation leads to destructive emphysema

abnormal pulmonary elastic fiber morphology ( J:205092 )

• alveolar elastic fiber organization and deposition are impaired

respiratory failure ( J:205092 )

• respiratory failure with postnatal mortality greater than 50%

cardiovascular system

abnormal lung vasculature morphology ( J:205092 )

• abnormal vascular development

• vascular changes consist of mild medial thickening and abnormalities in elastic fiber architecture

cellular

decreased lung endothelial cell proliferation ( J:205092 )

• cell proliferation is reduced in lung epithelia at the beginning of the alveolarization phase compared to controls which show an increase in proliferation

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
lower respiratory tract disease		DOID:0050161		J:205092

Genotype
MGI:5569821

tg2

• Allelic Composition: Tg(AGER)#Eca/0
• Genetic Background: C57BL/6J-Tg(AGER)#Eca

respiratory system

decreased lung endothelial cell proliferation ( J:205092 )

• cell proliferation is reduced in lung epithelia at the beginning of the alveolarization phase compared to controls which show an increase in proliferation

lung inflammation ( J:205092 )

• from 10-18 months of age, inflammatory cell infiltration is seen with loss of alveolar structures

abnormal lung morphology ( J:205092 )

• mice show an increase in the number of TUNEL-positive cells in the lungs at P4, P8, P20, and P30, indicating increased apoptosis

abnormal lung alveolus development ( J:205092 )

• altered alveolarization process and a reduction in secondary alveolar crest formation

abnormal pulmonary alveolar system morphology ( J:205092 )

• the volume density ratio of secondary alveolar crests/lung parenchyma in the lungs is lower than in controls, indicating a decrease in secondary crest volume density

• from 10-18 months of age, mice show progressive loss of alveolar structures

dilated pulmonary alveolar duct ( J:205092 )

• increase in size of alveolar ducts

overexpanded pulmonary alveolus ( J:205092 )

• lungs show diffuse airspace enlargement at P4

abnormal pulmonary interalveolar septum morphology ( J:205092 )

• lungs show short or absent secondary septa at P4

• increase in apoptosis in the alveolar septa

emphysema ( J:205092 )

• reduction of the number of alveolar septa and an increase in size of the alveolar ducts results in developmental emphysema

abnormal pulmonary elastic fiber morphology ( J:205092 )

• alveolar elastic fibers are fragmented, thin, not well-organized and distributed in the alveolar wall with an unraveled and loose appearance

immune system

lung inflammation ( J:205092 )

• from 10-18 months of age, inflammatory cell infiltration is seen with loss of alveolar structures

cellular

decreased lung endothelial cell proliferation ( J:205092 )

• cell proliferation is reduced in lung epithelia at the beginning of the alveolarization phase compared to controls which show an increase in proliferation