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Kudos to Dr. Young! Again!

Regular readers are well aware of the HPS Network’s relationship with the American Thoracic Society. We’ve partnered with them to award a research grant to Dr. Lisa Young for her work studying the pulmonary fibrosis of HPS.

Dr. Young just published a very interesting paper in the American Journal of Respiratory Cell and Molecular Biology titled: Susceptibility of Hermansky-Pudlak Mice to Bleomycin-Induced Type II Cell Apoptosis and Fibrosis.

Her findings were exciting in that they will help to further us along on the journey to nailing down exactly what happens in the lungs. The paper was also exciting because it is the first paper that credits the HPS Network as a research funder.

I’m a little leery to try to summarize some of Dr. Young’s findings for two reasons. I’m not a researcher and I could easily misunderstand something. Also, I know that when you’re reading things that are hard to understand, you tend to focus in on the things that make sense to you, and that isn’t always the most important part of the paper.

I will say this much, however. (And if any of our doctor/researcher types happen to read this, please feel free to correct any errors!!!!).


One of the challenges in researching HPS in mice is that mice only live about two years, and thus they don’t always develop all of the complications that humans develop. To speed up the fibrosis process in the mice, researchers studying pulmonary fibrosis often expose the mice to Bleomycin, a chemotherapy drug that accelerates the formation of fibrosis.

Dr. Young found several things. First, there’s something up with the lung cells of the mice way before fibrosis is ever on the scene. Second, when exposed to the Bleomycin, the HPS mice got sick way sooner than the regular, control group mice. In fact, she found it took half as much Bleomycin to make the HPS mice develop fibrosis as the regular mice. This is interesting because it provides clues about what exactly is going on with the cells in the lungs to cause the fibrosis. The paper looks at some of the differences observed between the lung tissue of the control group and the exposed HPS mice.

For so many years funding research was simply beyond what we could hope to do. We were lucky to raise enough money to pay the phone bill. While we’ve still got to keep up the hard work fundraising in order to keep paying those phone bills (and the translation bill) – it’s very exciting to have dipped our toes into the research pool.

And extra kudos to the Molters and Fort Wayne - were it not for them this grant wouldn't have been possible.


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