Research team

The innate immune system's role in cystic fibrosis

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Despite the fact that we breathe in 10,000 liters of air every day, which contains different types of particles and microorganisms, respiratory infections are relatively rare. This is because the lung harbors an effective immune system. Most bacteria found are commensal bacteria present in the airways without causing disease, but in immunocompromised individuals, these bacteria can get a foothold and cause serious infections. This is the case in individuals suffering cystic fibrosis (CF).

CF is an inherited disease characterized by repeated infections of the respiratory tract. The most common cause of death in CF is just the repeated infections leading to chronic inflammation and eventually to the destruction of the lungs. The infection often starts with staphylococcal infection that is maintained by influx of other opportunistic bacteria.

The body's immune cells and lung epithelial cells contribute to lung defense by having receptors (TLR and NLR) that detect the bacteria and then produce substances which control the inflammatory process. We have seen that CF cells have fewer receptors, indicating a deficient immune system, which in turn can lead to increased susceptibility to infection.