Idiopathic pulmonary fibrosis (IPF) is a progressive, lethal fibrotic lung disease. The focus of this research is elucidating the mechanisms underlying fibrotic progression. The researchers have identified a mesenchymal progenitor cell (MPC) in the lungs of IPF patients that give rise to fibrotic fibroblasts. The MPCs are isolated by flow cytometry using a SSEA4 antibody. RNA-seq analyses have shown that IPF MPCs are intrinsically different than MPCs islated from control patients. The researchers hypothesize that there are subpopulations of MPCs in the primary mesenchymal cell cultures from IPF patients. To examine this hypothesis they will perform single cell sequencing on SSEA4+ MPCs from control and IPF patients and analyze the data using MSI resources. They have also identified IPF mesenchymal cell subgroups and will analyze subgroup transcriptome by RNA-seq.
Additional studies will include generating IPF disease specific iPS cells which will then be differentiated to IPF mesenchymal stem cells (MSCs). In order to characterize the IPF MSCs derived from IPF iPS cells, the researchers will perform bulk RNA sequencing on these cells.