|
| Mouse and gene | Phenotypes | Reference |
|
| Tissue inhibitor of metalloproteinases-3 (TIMP-3) | Progressive emphysema from two weeks old with evidence of collagen degradation
and increased MMP activity | [56, 57] |
| Surfactant protein D (SP-D) | Progressive airspace enlargement with 3 weeks of life. Increased macrophages with
activated MMPs. The gene influences the response of alveolar epithelial type II cells
to the injurious events | [58, 59] |
| Lysosomal acid lipase (LAL) | LAL is a key enzyme in the metabolic pathway of neutral lipids. Areas of alveolar destruction because of neutrophil influx, foamy macrophages, and Clara cell hypertrophy | [60, 61] |
| Klotho | Klotho is an “antiageing” hormone and transmembrane protein | [62] |
| Integrin beta-6 (Itgb6) | Inhibition of TGF-β signaling causes increased expression of MMP-12 by macrophages. | [63] |
| Gamma retinoic acid receptor (RARγ) | Airspace enlargement because of impaired alveolar septation | [64, 65] |
| Platelet-derived growth factor A (PDGF-A) | Impaired alveolar septa lake of tropoelastin expression and lack lung alveolar
smooth muscle cells | [66] |
| Growth factor receptor 3 and 4(GFR 3-4) | Absence of secondary alveoli | [67] |
| Fibulin-5/DANCE | It is a secreted extracellular matrix protein that functions as a scaffold for elastin fiber assembly. The model is due to the interruption of elastin synthesis | [68, 69] |
| Elastin | Deficient formation of air sacs | [70] |
Tumor-necrosis alpha-converting
enzyme (TACE) | Disabled saccular structures | [71] |
| Adenosine deaminase | Increased adenosine levels impair alveolar septation and induce inflammation | [72, 73] |
| POD-1 | Hypoplastic lungs | [74] |
|
