A new study published in Science Translational Medicine reveals that cystic fibrosis (CF) causes alterations in the immune system early in life, possibly even in newborns. This discovery, made by an international research team including scientists from the Technical University of Munich (TUM), sheds light on why lung damage persists in CF patients despite new medications.

CF, a genetic disorder affecting the CFTR protein, primarily impacts the respiratory tract. While recent CFTR modulator therapies have improved patients' quality of life by reducing mucus formation, airway inflammation remains a persistent issue, especially in older patients.

The study found that blood samples from children with CF and biological material from pigs with the same genetic defect showed immature cells of the innate immune system. Senior author Nikolai Klymiuk explained, "We specifically looked at how the immune system behaves in cystic fibrosis before the cycle of infection and inflammation begins."

Search Antibodies
Search Now Use our Antibody Search Tool to find the right antibody for your research. Filter
by Type, Application, Reactivity, Host, Clonality, Conjugate/Tag, and Isotype.

The team observed an increased number and altered composition of immune cells in the lungs of newborn pigs with CF. Given the similarities between pig and human immune systems, this finding likely applies to human patients as well.

The team hypothesizes that these immune system changes could be due to an "emergency program" that rapidly produces a large number of immune cells, resulting in immature cells that contribute to the cycle of infections and inflammation in CF.

Importantly, these immune system alterations occur early in life and persist, contrary to previous beliefs that they were a consequence of numerous infections. The research suggests that addressing CF may require a multi-faceted approach beyond current CFTR modulator therapies.

Prof. Klymiuk concluded, "To enable people with cystic fibrosis to live without symptoms, we probably need to tackle the disease on several levels. We hope our work will help us better understand the causes of the defective immune system and correct them in the future."