Innate immune function defects

Pulmonary surfactant, a complex mixture of phospholipids and proteins synthesized and secreted by alveolar type 11 cells, functions to keep alveoli from collapsing during expiration (55). Surfactant is composed of 90% to 95% lipids and 5 to 10% proteins including SP-A, -B, -C, and -D (5,56,57). All four of the SP accumulate within the alveolar spaces in PAP (57). The two hydrophilic SP, A and D, have been the most studied. SP-A and SP-D belong to the coUectin family and play roles in the innate immunity of the lung (55). Pulmonary collectins have antimicrobial effects and display both inflammatory and anti-inflammatory properties (55). In iPAP, AMs exhibit defective clearance of surfactant however. 

NF-kB regulates both innate and adaptive immune responses ( immune defense) [2]. Understanding the function of NF-kB in the development, maintenance, and activation of cells from the immune system (including hematopoietic cells, macrophages, dendritic cells, B and T lymphocytes) has greatly benefited from the analysis of knockout mice in which individual NF-kB family members were defective. 

---