Phenotypes Associated with This Genotype

Genotype
MGI:5475008

• Allelic Composition: Tg(Msr1-MMP9)#Apar/0
• Genetic Background: B6.Cg-Tg(Msr1-MMP9)#Apar

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

respiratory system

abnormal pulmonary alveolar system morphology ( J:136830 )

• a marked decrease in elastin content is seen in alveolar regions of 1 year old mutants, with a decrease in the fractional volume area of fibrils within the alveolar walls

emphysema ( J:129959 , J:136830 )

• majority of mutants treated with bleomycin at 8-10 weeks of age exhibit emphysematous-like lesions in the lungs with ageing (at 18-24 weeks of age) (J:129959)

• mutants exhibit progressive air space enlargement, showing a progressive increase in the mean linear intercept after 2 months of age, indicating emphysema development (J:136830)

• the increase in mean linear intercept is equal to or greater than that reported in cigarette smoke-exposure model of emphysema (J:136830)

immune system

immune system phenotype ( J:136830 )

N • mutants do not show airway inflammation

abnormal macrophage physiology ( J:136830 )

• macrophages exhibit increased gelatinolytic activity

decreased inflammatory response ( J:129959 )

• mutants exhibit a different inflammatory response in the lungs in response to bleomycin than treated wild-type mice, showing a decrease in neutrophils and lymphocytes in bronchoalveolar lavage fluid compared to wild-type mice

• mutants develop less extensive pulmonary fibrosis at 8 weeks after bleomycin instillation than wild-type mice

• hydroxyproline, collagen, IGFBP3 and TIMP1 levels are significantly lower at 8 weeks after bleomycin instillation than wild-type mice

hematopoietic system

abnormal macrophage physiology ( J:136830 )

• macrophages exhibit increased gelatinolytic activity

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
pulmonary emphysema		DOID:9675	OMIM:130700	J:136830