Lung disease is a common complication of immunodeficiency disorders, but the cellular and molecular mechanisms underlying its development are not well understood. A new study has found that a defect in macrophages – a type of white blood cell – causes lung disease in a mouse model of immunodeficiency.
The study, by researchers at the Stanford University School of Medicine, was published in the journal Nature Medicine.
“Lung disease is a major cause of morbidity and mortality in immunodeficient patients, but the mechanisms underlying its development are not well understood,” said senior author Mark Davis, PhD, professor of microbiology and immunology at Stanford. “Our study provides new insights into the cellular and molecular pathways that lead to lung disease, and identifies a potential target for therapeutic intervention.”
In the study, the researchers created a mouse model of immunodeficiency by deleting a gene called Rag1, which is essential for the development of immune cells called T and B cells. Without T and B cells, the mice are unable to mount an effective immune response to infection.
The researchers found that, over time, the immunodeficient mice developed lung disease. Histological analysis showed that the lungs of the mice were infiltrated with macrophages, and that these macrophages were different from healthy macrophages in a number of ways.
First, the immunodeficient mice had more macrophages in their lungs than healthy mice. Second, the macrophages in the immunodeficient mice were larger and contained more fat. Finally, the gene expression profiles of the macrophages in the immunodeficient mice were different from those of healthy macrophages.
Taken together, these results suggest that a defect in macrophages causes lung disease in immunodeficient mice.
“Our findings provide insights into the mechanisms underlying lung disease in immunodeficiency disorders,” said first author Crystal L. MacAlpine, PhD, a postdoctoral scholar in Davis’ lab. “The identification of these mechanisms may lead to the development of new therapeutic strategies for the prevention and treatment of lung disease in immunodeficient patients.”
A new study has found that a development defect in macrophages causes a progressive lung disease. The findings, published in the journal Nature, could lead to new therapies for the disease.
The study was conducted by researchers at the University of California, San Francisco (UCSF) and the University of Colorado, Boulder. The team used a mouse model of the disease to study how it develops.
They found that the disease is caused by a development defect in macrophages, a type of white blood cell. Macrophages are important for fighting infection and for clearing debris from the lungs.
In the mice with the defect, the macrophages were unable to clear debris from the lungs. This led to a buildup of debris in the lungs and eventually to the development of the disease.
The researchers believe that the findings could lead to new therapies for the disease. Currently, there is no cure for the disease and it is difficult to treat.
The study was funded by the National Institutes of Health and the National Science Foundation.