An interdisciplinary team of DZL researchers from Hannover Medical School (MHH) at the DZL site BREATH has gained new insights into the role of so-called "aberrant basaloid cells" in idiopathic pulmonary fibrosis (IPF). The research findings help to understand how pathological changes in lung cells lead to the progressive scarring of lung tissue. The results were recently published in the American Journal of Respiratory Cell and Molecular Biology.
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 reduced oxygen uptake, significantly impairing the quality of life for those affected. In recent years, so-called aberrant basaloid cells have come into focus in IPF research. Through single-cell RNA sequencing, this new cell type has been described based on its transcription profile. They exhibit features of both lung epithelial cells and connective tissue cells, and seem to highly express many genes involved in the fibrosis process.
The Research Approach
To better understand the development and function of aberrant basaloid cells, an interdisciplinary team from the BREATH research group, 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 typical markers. They identified aberrant basaloid cells and then studied the exact same cells, including their immediate surroundings, in greater detail using electron microscopy. The results suggest that AE2 cells gradually develop into basaloid cells through intermediate stages.
Significance for the Future
The study shows that aberrant basaloid cells undergo intense remodeling processes, changing 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 down these processes. We hope to continue researching these cells and possibly find new therapeutic approaches," adds Dr. Schipke, her colleague.
Source: BREATH
Original publication:
Schipke J, Panpeng S, Wrede C, Hegermann J, Ruwisch J, Werlein C, Jonigk D, Shin HO, Schupp J, Mühlfeld C, Knudsen L. On the Ultrastructure of Aberrant Basaloid Cells in Idiopathic Pulmonary Fibrosis. Am J Respir Cell Mol Biol. 2024 Dec;71(6):746-750. doi: 10.1165/rcmb.2024-0218LE. PMID: 39601536.