Idiopathic Pulmonary Fibrosis and Aberrant Basaloid Cells

Idiopathic Pulmonary Fibrosis is a severe, chronic lung disease characterized by progressive scarring of lung tissue. This leads to shortness of breath and impaired oxygen uptake, which significantly reduces the quality of life for affected individuals. In recent years, so-called aberrant basaloid cells have moved into the focus of IPF research. Through single-cell RNA sequencing, this new cell type has been described based on its transcription profile. These cells exhibit characteristics of lung epithelial cells (surface tissue) and connective tissue cells and appear to highly express many genes involved in the fibrotic process.

The Research Approach

To better understand the development and function of aberrant basaloid cells, an interdisciplinary team of BREATH researchers led by PD Dr. Julia Schipke, Prof. Dr. Lars Knudsen, and Prof. Dr. Christian Mühlfeld analyzed explanted lungs diagnosed with IPF using immunofluorescence at the light microscopy level with specific markers. They identified aberrant basaloid cells and subsequently examined the exact same cells, including their immediate environment, in more detail using electron microscopy. The results suggest that AE2 cells gradually develop into basaloid cells through intermediate stages.

Significance for the Future

The study reveals that aberrant basaloid cells undergo intense remodeling processes in which they change their structure and function. They also lose their connections to the surrounding tissue, which may promote the progression of scarring. Additionally, they form layers of altered cells that further impair normal lung function.

"Our work shows that the pathological transformation of lung cells begins in the early stages of the disease," explains Dr. Lars Knudsen, one of the study's authors. "With this knowledge, we can more specifically search for ways to stop or slow these processes." Dr. Schipke adds, "We hope to continue our research on these cells and perhaps identify new therapeutic approaches."

The original publication can be found here.

Text: BREATH/AB

Photo: BREATH/AB