Chymotrypsin-like elastase 1 (CELA1) is a serine protease that is neutralized by alpha-1 antitrypsin (AAT) and prevents emphysema in a murine antisense oligonucleotide model of AAT-deficient emphysema. We tested the role of CELA1 in emphysema development in this genetic model of AAT-deficiency following tracheal lipopolysaccharide (LPS), 10 months of cigarette smoke (CS) exposure, aging, and a low-dose tracheal porcine pancreatic elastase (LD-PPE) model we developed. In this last model, we performed proteomic analysis to understand differences in lung protein composition. We were unable to show that AAT-deficient mice developed more emphysema than wild type with escalating doses of LPS.
[doi:10.25345/C5PG1HZ2N]
[dataset license: CC0 1.0 Universal (CC0 1.0)]
Keywords: lung ; alpha-1 antitrypsin deficiency ; mouse model
Principal Investigators: (in alphabetical order) |
Geremy Clair, Pacific Northwest National Laboratory, United States |
Submitting User: | alchemistmatt |
Devine AJ, Smith NJ, Joshi R, Fan Q, Borchers MT, Clair GC, Adkins JN, Varisco BM.
Chymotrypsin-like Elastase-1 Mediates Progressive Emphysema in Alpha-1 Antitrypsin Deficiency.
Chronic Obstr Pulm Dis. 2023 Oct 26;10(4):380-391.
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