Mouse Genome Informatics
cn
    Juntm4Wag/Juntm4Wag
Tg(Sftpc-cre)1Blh/0

involves: 129P2/OlaHsd * C57BL/6 * DBA/2
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
respiratory system
• mutants exhibit infiltration of atypical foamy alveolar macrophages
• mutants with prolonged exposure to cigarette smoke exhibit perivascular and peribronchiolar inflammation
• mutants exhibit an increase in apoptotic cells in the lungs
• 3 month old mutants exhibit enlarged air spaces and infiltration of atypical foamy alveolar macrophages
• progressive destruction of alveolar tissue
• mutants develop progressive emphysema
• localized destruction leading to large cavities in the parenchyma

immune system
• elevated levels of IL-12p40, IL-13, and CXCL1 cytokines in the BAL
• an elevated level of TNF-alpha is seen in the BAL fluid
• mutants exhibit infiltration of atypical foamy alveolar macrophages
• mutants with prolonged exposure to cigarette smoke exhibit perivascular and peribronchiolar inflammation

homeostasis/metabolism
• elevated levels of IL-12p40, IL-13, and CXCL1 cytokines in the BAL
• an elevated level of TNF-alpha is seen in the BAL fluid

Mouse Models of Human Disease
OMIM IDRef(s)
Emphysema, Hereditary Pulmonary 130700 J:179883
Pulmonary Disease, Chronic Obstructive; COPD 606963 J:179883