Mouse Genome Informatics
hm
    Sftpctm1Swg/Sftpctm1Swg
either: 129/Sv or (involves: 129/Sv * Black Swiss)
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       

Airspace remodeling, stromal thickening and monocytic infiltration in the lungs of Sftpctm1Swg/Sftpctm1Swg mice

mortality/aging
• fecundity was reduced by 2 months of age
• very few litters were produced after 6 months of age

behavior/neurological
• deteriorating coat condition after 2 months of age

growth/size
• 24% reduction in body weight by 1 year of age

immune system

reproductive system
• fecundity was reduced by 2 months of age
• very few litters were produced after 6 months of age

respiratory system
• alveolar capillary walls are thickened or obliterated by surrounding stroma and collagen
• epithelial cell dysplasia present by 6-12 months
• non ciliated columnar epithelia found with characteristics of Clara cells
• interstitial thickening of the lung parenchyma
• perivascular and peribronchiolar monocyte infiltration in severely affected individuals
• enlarged by 2 months
• irregular alveolar septation and shortened septal tips
• loss of alveolar elastin fibers
• fibrosis and collagen deposition at thickened alveolar septal structures
• some alveolar spaces obliterated by 12 months of age
• excessive lamellar bodies in type II cells
• hyperplasia of type II cells
• enlarged by 2 months
• irregular alveolar septation and shortened septal tips
• fibrosis and collagen deposition at thickened alveolar septal structures
• regions of severe emphysema with septal thinning and degeneration of pulmonary capillaries
• loss of alveolar elastin fibers
• airway resistance is increased

vision/eye

cardiovascular system
• alveolar capillary walls are thickened or obliterated by surrounding stroma and collagen

Mouse Models of Human Disease
OMIM IDRef(s)
Interstitial Pneumonitis, Desquamative, Familial; DIP 263000 J:82925