Jason Hong, M.D.

Pulmonary arterial hypertension (PAH) is a devastating and incurable disease characterized by abnormal narrowing of blood vessels within the lungs, ultimately leading to failure of the heart to pump blood to the lungs. A clear understanding of which cells and which genes are responsible for PAH is lacking but is critically needed to develop more effective therapies. We will use cutting-edge single cell techniques to better understand the behavior of the different types of cells that are found in the lungs and circulating blood of people with PAH compared to people without PAH. We will then use this knowledge to find and test an existing drug predicted by computer-based algorithms to reverse the abnormal behavior of the cell types most involved in PAH. Overall, this study will offer a more comprehensive understanding of the cell landscape in the lungs and blood of PAH patients and will identify potential drugs as novel therapies for PAH patients.

Supported by the Mary Fuller Russell Fund

Update:

We have now successfully performed an initial pilot experiment that was critical to ensure that we could isolate intact nuclei from human lung samples that had been archived for over two years. We also demonstrated that we could measure intact transcriptomes (the set of all RNA instructions) at single-nucleus resolution. To our knowledge, this is the first demonstration of successful single-cell sequencing on archived human lung samples in the PAH field.